THE  LIBRARY 

OF 

THE  UNIVERSITY 
OF  CALIFORNIA 

LOS  ANGELES 

GIFT  OF 


SAN  FRANCISCO 
COUNTY  MEDICAL  SOCIETY 


THE 
CASE  HISTORY  SERIES 


CASE   HISTORIES   IN   MEDICINE 

BY 

RICHARD  C.  CABOT,  M.D. 
Third  edition,  revised  and  enlarged 


CASE   HISTORIES   IN   PEDIATRICS 

BY 

JOHN  LOVETT  MORSE,  M.D. 
Second  edition,  revised  and  enlarged 


ONE   HUNDRED    SURGICAL   PROBLEMS 

BY 

JAMES  G.  MUMFORD,  M.D. 
Second  Printing 


CASE   HISTORIES   IN   NEUROLOGY 

BY 

E.   W.  TAYLOR,  M.D. 
Second  Printing 


CASE   HISTORIES   IN   OBSTETRICS 

BY 

ROBERT  L.  DENORMANDIE,  M.D. 

Second  Edition 


CASE  HISTORIES   IN   DISEASES   OF   WOMEN 

BY 

CHARLES  M.  GREEN,  M.D. 


NEUROSYPHILIS 

MODERN  SYSTEMATIC  DIAGNOSIS  AND  TREATMENT 
Presented  in  one  hundred  and  thirty-seven  Case  Histories 

BY 
E.  E.  SOUTHARD,  M.D.,  Sc.D. 


AND 


H.  C.  SOLOMON,  M.D. 

Being  Monograph  Number  Two  of  the  Psychopathic  Hospital,  Boston, 
Massachusetts.  (Monograph  Number  One  was  A  Point  Scale  for  Measur- 
ing Mental  Ability  by  Robert  M.  Yerkes,  James  W.  Bridges  and  Rose 
S.  Hardwick.  Published  by  Warwick  and  York.  Baltimore  1915.) 


METCHNIKOFF 
SCHAUDINN 


EHRLICH 


NEUROSYPHILIS 

MODERN  SYSTEMATIC  DIAGNOSIS  AND 
TREATMENT 

PRESENTED  IN  ONE  HUNDRED  AND  THIRTY- 
SEVEN  CASE  HISTORIES 


BY 
E.  E.  SOUTHARD,  M.D.,  Sc.D., 

Bullard  Professor  of  Neuropathology,  Harvard  Medical  School;    Pathologist,  Massachusetts 

Commission  on  Mental  Diseases ;  Director,  Psychopathic  Department, 

Boston  State  Hospital ;  Vice- President,  American 

Medico-Psychological  Association 

AND 

H.  C.  SOLOMON,  M.D., 

Instructor  in  Neuropathology  and  in  Psychiatry,  Harvard  Medical  School;    Special  Investi- 
gator in  Brain  Syphilis,  Massachusetts  Commission  on  Mental  Diseases ;    Acting 
Chief-of-Staff,  Psychopathic  Department,  Boston  State  Hospital 


WITH  AN  INTRODUCTION  BY 
JAMES  JACKSON  PUTNAM,  M.D., 

Profenor  Emeritu*  of  Dneaie*  of  the  Nerroui  System,  Harvard  Medical  School 


BY  VOTE  Ot  THE  TRUSTEES  OT  THE  BOSTON  STATE  HOSPITAL 

MONOGRAPH  NUMBER  TWO 

OF  THE 

PSYCHOPATHIC  HOSPITAL,  BOSTON,  MASSACHUSETTS 


BOSTON 

W.  M.  LEONARD,  PUBLISHER 
1917 


Copyright,  1917, 
By  W.  M.  Leonard 


we 


MASSACHUSETTS 

A  STATE  THAT 

BOTH  TOLERATES  AND  FOSTERS 
RESEARCH 


PREFACE 

THIS  book  is  written  primarily  for  the  general  practi- 
tioner and  secondarily  for  the  syphilographer,  the  neurologist, 
and  the  psychiatrist.  Our  material  is  drawn  chiefly  from  a 
psychopathic  hospital,  that  modern  type  of  institution  in 
which  the  mental  problems  of  general  medical  practice  come 
to  a  diagnostic  head  weeks,  months,  or  years  before  the 
asylum  is  thought  of. 

It  is  this  peculiar  nature  of  psychopathic  hospital  material 
—  a  concentrated  essence  of  the  most  difficult  daily  problems 
of  general  practice  —  that  brings  together  such  an  apparent 
melange  of  cases  as  are  here  described,  ranging  from  mild 
single-symptom  diseases  like  extraocular  palsy  up  to  genuine 
magazines  of  symptoms  as  in  general  paresis;  from  feeble- 
mindedness, apparently  simple,  up  to  apparently  simple 
dotage,  both  feeble-mindedness  and  dotage  really  syphi- 
litic; from  the  mind-clear  tabetic  to  the  maniacal  or  deluded 
subject  who  looks  physically  perfectly  fit;  from  the  early 
secondaries  to  the  late  tertiaries  or  so-called  quaternaries; 
from  peracute  to  the  most  chronic  of  known  conditions; 
from  the  most  delicate  character  changes  to  the  profoundest 
ruin  of  the  psyche. 

Although  the  bulk  of  our  case-material  is  drawn  from  gen- 
eral practice  through  the  thinnest  of  intermediary  membranes, 
the  psychopathic  hospital,  yet  we  have  tried  to  depict  the 
whole  story  by  presenting  enough  autopsied  cases  from  district 
state  hospitals  to  show  exactly  what  treatment  has  to  face. 
Nor  have  we  hesitated  to  insert  cases  in  which  treatment 
has  failed. 

In  addition  to  (a)  the  Psychopathic  Hospital,  Boston, 
group  of  incipient,  doubtful,  obscure,  or  complicated  cases 
(the  early  clinical  group)  and  (b)  the  Danvers  State  Hospital, 
Hathorne,  group  of  longer-standing,  committed,  fatal  cases 

5 


6  PREFACE 

(the  finished  or  autopsied  group)  we  present  (c)  a  miscel- 
laneous group  of  cases,  including  many  from  private  neuro- 
logical or  psychiatric  practice.  No  doubt  those  familiar  with 
Boston  medicine  will  see  traces  of  the  teaching  of  our  former 
chiefs,  notably  Professors  James  Jackson  Putnam  and  Ed- 
ward Wyllys  Taylor.  We  are  obliged  to  them  for  some  well- 
observed  cases. 

We  have  dedicated  our  work  to  the  Commonwealth,  but 
perhaps  we  should  more  specifically  ascribe  to  the  Massa- 
chusetts Commission  on  Mental  Diseases  (formerly  the 
State  Board  of  Insanity)  the  spirit  that  permitted  our  special 
study  of  neurosyphilis  treatment.  To  these  authorities, 
who  have  countenanced  and  encouraged  a  somewhat  costly 
piece  of  special  work  since  1914,  we  offer  our  thanks,  hoping 
that  other  states  will  be  one  by  one  stimulated  to  the  state- 
endowment  of  research.  States  doing  full  duty  by  research 
can  be  counted  on  one  hand. 

To  our  Psychopathic  Hospital  colleagues  and  the  internes, 
and  especially  to  Drs.  Myrtelle  M.  Canavan  and  Douglas 
A.  Thorn  of  the  Commission's  Pathological  Service,  we  also 
offer  our  best  thanks. 

The  Danvers  traditions  are  tangible  here:  cases  of  Drs.  A. 
M.  Barrett,  H.  A.  Cotton,  H.  W.  Mitchell,  H.  M.  Swift,  and 
others  are  presented.  We  have  been  especially  aided  by  the 
more  recent  work  of  Dr.  Lawson  G.  Lowrey. 

Nor  should  we  have  been  able  to  present  our  samples 'of 
brain  correlation  without  drawing  on  the  collection  arranged 
and  analyzed  by  Dr.  Annie  E.  Taft,  Custodian,  Harvard  De- 
partment of  Neuropathology.  The  photographs,  part  of  a 
collection  of  brain  photographs  now  numbering  over  10,000 
representing  700  brains  of  all  sorts,  were  made  by  Mr.  Herbert 
W.  Taylor. 

The  Wassermann  testing  work  has  been  done  by  Dr.  W. 
A.  Hinton  of  the  State  Board  of  Health.  Dr.  Hinton  himself 
wrote  out  the  text  description  of  the  Wassermann  method. 
The  method  of  his  laboratory  is  held  to  the  standards  of  con- 
trol set  by  previous  chiefs,  viz.  by  Professor  F.  P.  Gay,  who 
brought  immunological  methods  direct  from  the  laboratory 
of  Bordet  (whose  method  the  Wassermann  method  essentially 


PREFACE  7 

is),  Prof.  W.  P.  Lucas,  and  the  late  Dr.  Emma  W.  D.  Mooers, 
who  had  assisted  Plaut  in  his  first  work  with  the  Wassermann 
method  in  Kraepelin's  Munich  Clinic. 

The  material  combed  by  us  to  secure  this  illustrative  series 
amounts  to  over  2000  cases  of  syphilis  of  the  nervous  system, 
including  over  100  autopsies  in  all  types  of  case.  We  have 
presented  these  with  very  varying  fulness,  chiefly  to  illustrate 
the  contentions  at  the  heads  of  the  case-descriptions. 

In  using  the  book,  we  suggest  early  reference  to  the  Sum- 
mary and  Key,  where  for  convenience  are  placed  numerous 
cross-references  permitting  extended  illustration  of  almost 
every  proposition  from  several  cases. 

We  have  not  made  a  large  feature  of  the  Medicolegal  and 
Social  section.  This  kind  of  thing  well  deserves  a  volume  by 
itself,  with  all  the  legal  and  social-service  implications  drawn 
out  in  their  amazing  richness  and  detail.  The  social  service 
slogan,  "A  paretic's  child  is  a  syphilitic's  child"  has  already 
accomplished  a  great  deal  of  good  in  our  local  world.  -  Some 
day  we  may  not  be  compelled  to  drive  the  paretic's  spouse  and 
offspring  to  the  Wassermann  serum  test!  The  general  prac- 
titioner must  help  here. 

A  note  on  the  Treatment  section.  This  is  manifestly  not 
the  last  word  or  even,  we  hope,  our  own  last  word,  since  the 
systematic  work  of  the  Massachusetts  Commission  must  be 
kept  up  for  some  years  to  get  a  reliable  verdict.  Some  of 
the  results  give  rise  to  greater  optimism  than  has  prevailed 
in  asylum  circles,  especially  re  general  paresis.  We  are  con- 
fident that  no  one  can  now  successfully  make  a  differential 
diagnosis  between  the  paretic  and  the  diffuse  non-paretic  forms 
of  neuro syphilis  in  many  phases  of  either  disease,  even  with  all 
laboratory  refinements.  If  this  be  so,  it  is  improper  not  to 
give  the  full  benefits  of  modern  treatment  to  all  cases  in  which 
the  diagnosis  remains  doubtful  between  the  paretic  and  the 
diffuse  non-paretic  forms  of  neurosyphilis.  We  ourselves  ad- 
vocate modern  treatment,  not  only  in  the  diffuse,  but  also 
in  early  paretic  forms  of  neurosyphilis. 

It  would  have  been  out  of  place  in  a  book  in  this  Case 
History  Series  to  have  dealt  extensively  with  the  history  of 
our  topic.  We  have  compensated  inadequately  for  this  lack 


8  PREFACE 

by  a  few  remarks  at  the  head  of  the  Summary  and  Key. 
We  are,  like  all  others  in  the  field,  under  the  inevitable  obli- 
gation to  Nonne  of  Hamburg,  whose  great  work  has  gone  into 
three  editions,  the  second  of  which  has  appeared  in  English 
translation  (Nonne's  Syphilis  of  the  Nervous  System,  C.  R. 
Ball,  translator).  Mott's  work,  embodied  in  a  large  volume 
of  the  ^Power-Murphy  System  of  Syphilis,  has  also  been  at- 
tentively consulted,  as  well  as  the  various  systematic  works 
on  neurology  and  psychiatry.  The  topic  of  Neurosyphilis  is 
getting  wide  and  appropriate  attention  in  this  country  through 
special  journals,  both  those  dealing  with  nervous  and  mental 
diseases,  and  those  dealing  with  syphilis.  Syphilis  is  in  a 
sense  the  making  of  psychiatry  and  will  go  far  to  pushing 
psychiatry  into  general  practice. 

At  the  last  moment  we  have  been  led  to  deviate  from  our 
plan  of  presenting  only  local  cases  familiar  and  accessible  to 
us.  In  a  section  on  Neurosyphilis  and  the  War,  we  present 
excerpts  and  digests  of  English,  French,  and  German  cases 
of  neurosyphilis  that  have  appeared  in  association  with  the 
war.  Our  own  country  has  not  suffered  greatly  as  yet  either 
from  the  lighting  up  of  neurosyphilis  under  martial  stress  or 
from  the  immediate  or  remote  effects  of  syphilis  obtained 
in  the  unholy  congress  of  Mars  and  Venus.  Space  forbids  a 
large  collection  of  these  martial  cases,  but,  as  will  be  seen,  a 
fair  sample  of  problems  is  presented. 

Speaking  for  the  moment  as  the  senior  author  of  this  book, 
I  wish  to  say  that,  were  it  not  for  the  energy,  industry,  and 
ingenuity  of  the  junior  author,  Dr.  H.  C.  Solomon,  the  book 
would  not  have  been  written.  Nor,  in  all  probability,  would 
the  systematic  work  of  the  Commonwealth  on  neurosyphilis 
and  its  treatment  ever  have  been  begun.  I  can  also  accord 
the  highest  praise  to  Mrs.  Maida  Herman  Solomon  for  her 
social-service  work  in  this  new  field. 

Perhaps,  in  closing,  we  owe  an  apology  to  John  Milton  for 
our  borrowings  from  the  two  Paradises.  Had  he  known 
much  about  syphilis,  Milton  might  have  written  still  stronger 
mottoes  for  us. 

E.  E.  SOUTHARD 

74  KENWOOD  ROAD 
Boston,  Massachusetts 


TABLE  OF  CONTENTS 

PAGE 

SECTION  I.  THE  NATURE  AND  FORMS  OF  SYPHILIS  OF  THE  NERVOUS  SYS- 
TEM (NEUROSYPHILIS).    CASES  i  TO  8 17 

CASE 

1.  Paradigm:   protean  symptoms,  nervous  and  mental.     Autopsy,  with 

meningeal,  parenchymatous,  and  vascular  lesions 17 

2.  Tabes  dorsalis  (tabetic  neurosyphilis).     Autopsy 31 

3.  General  paresis  (paretic  neurosyphilis).     Autopsy 37 

4.  Cerebral  thrombosis  (vascular  neurosyphilis).     Autopsy 42 

5.  Juvenile  paresis  (juvenile  paretic  neurosyphilis).     Autopsy 45 

6.  Extraocular  palsy  (focal  meningeal  neurosyphilis).     Autopsy 50 

7.  Gumma  of  brain  (gummatous  neurosyphilis).     Autopsy 53 

8.  Meningitis     hypertrophica  tcervicalis     (gummatous  .  neurosyphilis). 

Autopsy 56 

SECTION  II.  THE  SYSTEMATIC  DIAGNOSIS  OF  THE  FORMS  OF  NEUROSYPHILIS. 

CASES  9  TO  38 63 

CASE 

9.  Neurasthenia  versus  neurosyphilis 63 

10.  Paretic  neurosyphilis  versus  manic-depressive  psychosis 68 

11.  Neurosyphilis  versus  manic-depressive  psychosis 71 

12.  Dementia  praecox  versus  neurosyphilis.     Autopsy 74 

13.  Neurosyphilis:  negative  Wassermann  reaction  (W.  R.)  of  serum 77 

14.  Diffuse  neurosyphilis:  six  tests  apt  to  run  mild 80 

15.  Paretic  neurosyphilis:  six  tests  strong 85 

16.  Taboparesis  (tabetic  neurosyphilis):  tests  like  those  of  paresis 92 

,17.    Paretic  versus  diffuse  neurosyphilis:  confusion  re  tests 97 

18.  [Vascular  neurosyphilis:  positive  serum,  negative  fluid  W.  R 101 

19.  Seizures  in  diffuse  neurosyphilis 103 

20.  Seizures  in  paretic  neurosyphilis 106 

21.  Aphasia  in  paretic  neurosyphilis in 

22.  Aphasia  in  paretic  neurosyphilis 115 

23.  Remission  in  paretic  neurosyphilis 117 

24.  Remission  in  diffuse  neurosyphilis 122 

25.  Paresis  sine  paresi 126 

26.  Paretic  neurosyphilis.     Autopsy 131 

27.  Gummatous  neurosyphilis.     Operation 137 

28.  Extraocular  palsy  (cranial  neurosyphilis) 140 

29.  Tabes  dorsalis  (tabetic  neurosyphilis) :  six  tests  apt  to  run  mild 141 

30.  Tabetic  neurosyphilis,  clinically  atypical 143 

31.  Cervical  tabes 146 

32.  Erb's  syphilitic  spastic  paraplegia 147 

33.  Syphilitic  muscular  atrophy 149 

9 


Io  CONTENTS 

CASE  PAGE 

34.  Neurosyphilis  of  the  secondary  period 

35.  Juvenile  paretic  neurosyphilis:  optic  atrophy 154 

36.  Juvenile  paretic  neurosyphilis 157 

37.  Simple  feeblemindedness,  syphilitic 159 

38.  Juvenile  tabes l61 

SECTION  III.   PUZZLES  AND  ERRORS  IN  THE  DIAGNOSIS  OF  NEUROSYPHILIS 

(INCLUDING  NON-SYPHILITIC  CASES).    CASES  39-82 165 

CASE 

39.  Paretic  versus  diffuse  neurosyphilis.     Autopsy 165 

40.  Paretic  versus  vascular  neurosyphilis,  cerebellar.     Autopsy 169 

41.  Paretic  versus  vascular  neurosyphilis,  cerebellar.     Autopsy 172 

42.  Tabetic  combined  with  vascular  neurosyphilis.     Autopsy 175 

43.  Tabetic  neurosyphilis:  mental  symptoms,  non-paretic.     Autopsy....  177 

44.  Cerebral  gliosis.     Autopsy 180 

45.  Neurasthenia  versus  neurosyphilis 183 

46.  Hysteria.     Neurosyphilis  of  the  secondary  period 185 

47.  Manic-depressive  psychosis  versus  paretic  neurosyphilis 187 

48.  Cerebral  tumor 190 

49.  Early  post-infective  paretic  neurosyphilis 192 

50.  Atypical  paretic  neurosyphilis,  hemitremor.     Autopsy 197 

51.  Paretic  neurosyphilis.     Autopsy 199 

52.  Manic-depressive  psychosis  versus  paretic  neurosyphilis 202 

53.  Syphilitic  (?)  exophthalmic  goitre.     Autopsy 205 

54.  Argyll-Robertson  pupils 209 

55.  Argyll- Robertson  pupils:  pineal  tumor.     Autopsy 212 

56.  Neurosyphilis  (?)  with  negative  spinal  fluid 216 

57.  Disseminated  syphilitic  encephalitis,   seven   months  post-infective. 

Autopsy 218 

58.  "  Pseudoparesis  " 222 

59.  Syphilitic  paranoia? 225 

60.  Paretic  neurosyphilis  versus  alcoholic  pseudoparesis 227 

61.  Alcoholic  pseudoparesis  versus  paretic  neurosyphilis 231 

62.  Alcoholic  neuritis  and  paretic  neurosyphilis 234 

63.  Chronic  alcoholism  versus  paretic  neurosyphilis 236 

64.  Neurosyphilis,  diabetic  pseudoparesis,  or  brain  tumor 238 

65.  Neurosyphilis  and  diabetes 240 

66.  Neurosyphilis:  hemianopsia 242 

67.  Paretic  neurosyphilis  versus  syphilis  and  cerebral  malaria 245 

68.  Paretic  neurosyphilis:  gold  sol  test  "syphilitic."     Autopsy 247 

69.  Lues  maligna 250 

70.  Neurosyphilis  versus  multiple  sclerosis 253 

71.  Atypical  neurosyphilis 256 

72.  Huntington's  chorea  versus  neurosyphilis 258 

73.  Senile  arteriosclerotic  psychosis  versus  neurosyphilis 262 

74.  Hysterical  fugue  versus  neurosyphilis 264 

75.  Tabetic  neurosyphilis  versus  pernicious  anemia 267 

76.  Congenital  neurosyphilis 270 

77.  Congenital  versus  paretic  neurosyphilis 272 

78.  Juvenile  paretic  neurosyphilis 275 


CONTENTS  II 

CASE  PAGE 

79.  Epilepsy  versus  juvenile  neurosyphilis 277 

80.  Addison's  disease  and  juvenile  paretic  neurosyphilis.  t  Autopsy 279 

81.  Neurosyphilis  of  the  secondary  period 283 

82.  Taboparetic  neurosyphilis  and  typhoid  meningitis.     Autopsy 284 

SECTION  IV.  NEUROSYPHILIS,  MEDICOLEGAL  AND  SOCIAL.  _CASES  83-98 . . .  289 
CASE 

83.  A  public  character,  neurosyphilitic.    Autopsy 289 

84.  Debts,  neurosyphilitic 295 

85.  Suicidal  attempt  by  a  neurosyphilitic 296 

86.  Neurosyphilis  and  juvenile  delinquency 298 

87.  Neurosyphilis  in  a  defective  delinquent 300 

88.  Paresis  sine  paresi  in  a  forger 303 

89.  Trauma:  juvenile  paretic  neurosyphilis 306 

90.  Trauma:  paretic  neurosyphilis 308 

91.  False  claim  for  trauma:  neurosyphilis 309 

92.  Traumatic  exacerbation?     in  neurosyphilis 310 

93.  Trauma:  cranial  gumma  at  the  site  of  injury 311 

94.  Occupation-neurosis  versus  syphilitic  neuritis 312 

95.  Character  change:  neurosyphilis 314 

96.  A  neurosyphilitic  family 316 

97.  A  neurosyphilitic's  normal-looking  family 318 

98.  The  neurosyphilitic's  marriage 319 


SECTION  V.  THE  TREATMENT  OF  NEUROSYPHILIS.    CASES  99-123. 

(CASES  99-103  SHOW  THE  VARIETY  OF  STRUCTURAL  LESIONS  THAT 

TREATMENT  HAS  TO  FACE) 323 

CASE 

99.   An  incurable  spastic  paresis  in  paretic  neurosyphilis.     Autopsy 323 

100.  A  theoretically  curable  case.     Autopsy 328 

101.  A    highly    meningitic    case,    theoretically    amenable    to    treatment. 

Autopsy 332 

102.  A  highly  atrophic  case,  theoretically  not  amenable  to  treatment. 

Autopsy 335 

103.  Paretic  neurosyphilis  with  markedly  focal  lesions.     Autopsy 338 

(CASES  104  TO  123  ARE  EXAMPLES  OF  TREATMENT  INCLUDING  SUC- 
CESSES AND  FAILURES.) 

104.  Diffuse  neurosyphilis:   treatment  successful  after  nine  months 342 

105.  Atypical  neurosyphilis:  treatment  successful 346 

106.  Argyll- Robertson  pupil  not  necessarily  of  bad  prognosis:  treated  case 

an  insurance  risk 350 

107.  Spinal  fluid  cleared:  symptoms  persistent 355 

108.  Arteriosclerosis  does  not  contraindicate  treatment 359 

109.  Symptoms  of  intracranial  pressure  relieved  by  treatment 362 

no.   Therapeutic  improvement  in  tabetic  neurosyphilis 366 

in.   W.  R.  rendered  negative  in  tabetic  neurosyphilis 367 

112.  Example  of  successful  treatment  of  paretic  neurosyphilis 37° 

113.  Another  example 372 


12  CONTENTS 

CASE  PAGE 

114.  Clinical  recovery  but  tests  persistently  positive  in  treated  paretic 

neurosyphilis 375 

115.  Improvement  delayed  in  treated  paretic  neurosyphilis 377 

116.  Non-neural  syphilis  in  treated  paretic  neurosyphilis 380 

117.  Partial  recovery  in  treated  paretic  neurosyphilis 382 

11 8.  Laboratory  signs  improved:    clinical  situation  stationary:    treated 

paretic  neurosyphilis 384 

119.  Another  example 386 

120.  Failure  of  treatment 388 

121.  Treatment,  at  first  mild,  later  intensive 390 

122.  Intensive  treatment 392 

123.  Syphilitic  feeblemindedness  improved  by  treatment 395 

SECTION  VI.  NEUROSYPHILIS  AND  THE  WAR. 

CASES  A  TO  N  FROM  BRITISH,  FRENCH,  AND  GERMAN  WRITERS 

(1914-1916) 
CASE 

A.  Tabes  "shell-shocked"  into  paresis?     (Donath) 401 

B.  Latent  syphilis  "shell-shocked"  into  tabes?     (Duco  and  Blum) ....  403 

C.  Aggravation  of  neurosyphilis  by  service?     (Weygandt) 404 

D.  Aggravation  of  neurosyphilis  by  service?     (Todd) 406 

E.  Aggravation  of  neurosyphilis  on  service?     (Todd) 409 

F.  Duration  of  neurosyphilitic  process  important.     (Farrar) 411 

G.  Latent  syphilis  lighted  up  to  paresis  by  war  stress  without  shell- 

shock.  (Marie) 412 

H.  Paresis  lighted  up  by  "gassing"?  (de  Massary) 414 

I.  Epilepsy  in  a  neuropath  lighted  up  by  syphilis  acquired  at  war. 

(Bonhoeffer) 415 

J.  Syphilitic  —  after  Dixmude  epileptic.  (Bonhoeffer) 417 

K.  Syphilitic  root-sciatica  in  a  fire-works  man.  (Dejerine,  Long) 418 

L.  Paresis  lighted  up  in  civilian  by  domestic  stress  of  the  war.  (Percy 

Smith) 420 

M.  Shell-shock  pseudoparesis.  (Pitres  and  Marchand) 421 

N.  Shell-shock  pseudotabes.  (Pitres  and  Marchand) 424 

SECTION  VII.  SUMMARY  AND  KEY 427 

APPENDICES: 

A.  The  six  tests 471 

B.  Common  methods  of  treatment 486 


INTRODUCTION 

IT  is  a  privilege  to  be  allowed  to  write  a  word  of  introduc- 
tion to  a  textbook  which  so  richly  fulfils  its  function  as  does 
this  volume  on  the  manifold  disorders  classified  under  N euro- 
syphilis,  a  subject  of  which  the  importance  for  the  welfare  of 
society  is  found  to  loom  the  larger  the  more  deeply  its  mys- 
teries are  probed. 

The  case-histories  with  which  its  pages  are  so  amply 
stocked  are  carefully  analyzed  in  accordance  with  a  broadly 
chosen  plan,  and  the  generalizations  that  precede  and  follow 
them  are  obviously  based  on  a  wide  and  varied  personal 
experience  such  as  alone  could  render  a  familiarity  with  the 
literature  of  the  subjects  treated  adequate  to  its  best  useful- 
ness. Both  writers  were  indeed  well  adapted  for  this  task. 
Dr.  Southard,  as  everyone  is  aware,  has  long  been  a  highly 
conscientious,  ardent  and  productive  worker  in  the  depart- 
ment of  pathological  anatomy,  and  of  late  years  a  careful 
student  of  clinical  diagnosis  and  methods,  both  at  the  Danvers 
State  Hospital  and  still  more,  at  the  Psychopathic  Hospital 
which  he  worked  so  hard  to  found;  while  Dr.  Solomon's 
researches,  in  the  special  field  of  neurosyphilis,  have  been  of 
the  highest  order. 

Undoubted  as  are  the  merits  of  the  case-system  of  in- 
struction that  has  been  so  much  in  vogue  in  recent  years, 
and  excellent  as  is  the  modern  supplementation  of  this  method 
by  the  use  of  published  records,  the  danger  is  still  real  that 
the  student  will  have  presented  to  him  a  picture  of  nature  in 
disease  that  is  too  diagrammatic,  too  concise,  with  the  result 
that  while  the  task  of  memory  is  lightened  through  simplified 
formulation,  the  training  of  the  doubting  and  inquiring 
instincts  is  often  given  too  little  stimulus  and  scope.  In 
this  book  this  danger  is  deliberately  met  through  the  cast- 
ing of  emphasis  rather  on  the  pluralistic  aspects  of  the  pro- 
cesses at  stake  than  (primarily)  on  their  unitary  aspects. 

13 


14  INTRODUCTION 

The  student  who  utilizes  this  volume  cannot  but  emerge 
from  his  study  a  more  thoughtful  person  than  he  was  at  the 
period  of  his  entry.  He  will  have  seen  that  clinical  rules  of 
thumb  cannot  be  followed  to  advantage,  and  that,  on  the 
contrary,  surprises  are  to  be  expected  and  prepared  for. 
Let  the  recognition  of  this  fact,  if  it  seems  to  increase  the 
difficulties  in  the  way  of  diagnosis,  not  lead  to  pessimism  in 
that  respect,  or  to  hopelessness  in  therapeutics.  On  the 
contrary  the  writers'  bias  is  towards  the  worth-whileness  of 
clinical  efforts  and  an  increased  respect  for  accuracy  and 
thoroughness  in  the  utilization  of  modern  methods  of  research. 
The  chance  is  indeed  held  open  that  even  the  gaunt  spectre 
of  "General  Paresis"  may  prove  to  be  less  terrible  than  it 
seems,  and  for  this  hope  good  grounds  are  given. 

It  is  in  this  way  made  clear,  on  the  strength  of  anatomical 
evidence  of  much  interest,  that  even  if  in  the  treatment  of  a 
given  patient,  the  time  arrives  when  a  fatal  or  unfavorable 
result  seems  manifestly  foreshadowed,  it  may  be  still  worth 
while  to  renew  the  treatment  with  fresh  zeal,  for  the  sake  of 
combatting  some  symptom  or  exacerbation,  for  which  a 
locally  fresh  process  furnishes  the  cause. 

Another  noteworthy  principle  here  emphasized  and  illus- 
trated is  that  the  relationship  between  "functional"  (hys- 
terical, neurasthenic,  migrainoid)  symptoms  and  the  signs 
(or  symptoms)  of  organic  processes  is  clinically  important 
and  worthy  of  much  further  study.  This  is  a  matter  which, 
in  a  general  sense,  has  interested  me  for  many  years.  Above 
and  over  the  "organic"  hovers  always  the  "functional,"  as 
representing  the  first  indication  of  the  marvelous  tendency 
to  repair,  or  substitution,  for  which  the  resources  of  nature 
are  so  vast.  Yet  this  functional  tendency  also  has  its  laws, 
of  which,  in  their  turn,  the  organic  processes  display  the 
action  in  quasi  diagrammatic  form.  Hysteria,  neurasthenia, 
migraine,  etc.,  do  not  arise  de  novo  in  each  case,  but  conform 
to  typical,  though  not  rigid,  formulas,  susceptible  of  descrip- 
tion. I  have  recently  had  the  opportunity  to  study  in  detail 
an  analogous  series  of  transitions  between  the  movements 
(and  emotions)  indicative  of  apparently  purposeless  myo- 
clonic  movements  (on  an  epileptoid  basis)  and  the  movements 


INTRODUCTION  15 

of  surprise,  engrossment,  purposeful  effort,  the  excitement 
and  joy  by  which  the  former  were  excited  and  into  which 
they  shaded  over. 

Taken  altogether,  this  book  represents  work  and  thought 
in  which,  for  amount  and  kind,  the  neurologists  of  Boston 
may  take  just  pride. 

JAMES  J.  PUTNAM. 

ST.  HUBERT'S,  KEENE  VALLEY,  NEW  YORK. 
August,  1917. 


Me  miserable!  which  way  shall  I  fly 
Infinite  wrath  and  infinite  despair? 
Which  way  I  fly  is  Hell;  myself  am  Hell; 
And,  in  the  lowest  deep,  a  lower  deep 
Still  threatening  to  devour  me  opens  wide, 
To  which  the  Hell  I  suffer  seems  a  Heaven. 


Paradise  Lost,  Book  IV,  lines  73-78. 


I.  THE  NATURE  AND  FORMS  OF  SYPHILIS  OF 
THE  NERVOUS  SYSTEM  (NEUROSYPHILIS) 


PARADIGM    to    show   possible    abundance    and 
variety   of   symptoms   and   lesions   in   DIFFUSE 
NEUROSYPHILIS  ^  ("  cerebrospinal    syphilis"). 
Autopsy. 


Case  i.  Mrs.  Alice  Morton*  was  in  the  hands  of  at  least 
five  well-known  specialists  in  different  branches  of  medicine 
and  surgery  during  the  nineteen  years  of  her  disease.  It 
appears  that  she  acquired  syphilis  upon  marriage  at  the  age 
of  23  to  a  man  who  later  became  tabetic  and  acknowledged 
syphilitic  infection  previous  to  marriage.  Mrs.  Morton 
remained  without  children  and  there  were  no  miscarriages. 

At  the  age  of  27,  she  developed  iritis,  paresis  of  the  left  eye 
muscles,  and  ulceration  of  the  throat,  with  destruction  of  the 
uvula.  The  syphilitic  nature  of  her  disease  was  at  once 
recognized  and  the  classical  treatment  was  given,  although, 
through  numerous  shifts  in  consultants,  this  treatment  was 
never  pushed  to  the  limit.  At  28  Mrs.  M.  began  to  suffer 
from  severe  headaches  resembling  migraine  and  accompanied 
by  attacks  of  panesthesia;  at  35,  came  severe  pains  in  the 
back  and  difficulty  in  walking. 

At  36,  the  migraine  attacks  began  to  be  accompanied  by 
blurring  of  vision  and  dizziness.  The  difficulty  in  walking 
became  extreme,  affecting  particularly  the  right  foot.  The 

*  The  cases  chosen  to  illustrate  the  propositions  of  the 
boxed  headings  always  illustrate  several  other  points.  See 
the  footnotes  of  Section  VI  for  lists  of  cases  illustrating 
special  points.  The  names  assigned  to  the  cases  are  fictitious 
and  chosen  to  suggest  race  or  descent. 


1 8  FORMS  OF  NEUROSYPHILIS 

legs  became  spastic,  there  were  pains  and  hypersesthesia  of 
the  chest,  and  severe  cramps  of  the  legs.  Anti-syphilitic 
treatment  at  this  time  yielded  marked  improvement. 

During  her  thirty-sixth  year,  Mrs.  M.  sustained  curious 
transient  losses  of  vision  and  of  hearing.  She  was  also  ir- 
ritable, and  at  this  time  developed  her  first  pronounced  mental 
symptoms,  namely,  delusions  concerning  her  relatives. 
There  were  also  a  few  seizures  of  an  epileptiform  nature. 
At  38  there  was  a  spell  of  total  deafness,  followed  by  im- 
provement. The  eye  muscles  were  also  subject  to  a  varia- 
ble involvement  with  intervening  spells  of  improvement. 
The  knee-jerks  were  lost,  but]  after  a  time  returned  in  less 
pronounced  form.  Shortly,  an  absolute  paralysis  and  ex- 
tensive decubitus  developed,  and  death  occurred  at  39. 

The  autopsy  is  briefly  summarized  below,  but  it  is  important 
in  the  understanding  of  Mrs.  M.'s  case  (particularly  some  of 
the  sensory  symptoms  and  the  transiency  of  certain  symptoms) 
to  consider  the  pre-infective  history.  Although  there  seems 
to  be  no  doubt  that  the  patient  acquired  syphilis  at  about 
23  years  of  age  from  a  syphilitic  husband,  who  himself  later 
became  tabetic,  yet  it  is  of  note  that  the  patient  was  the 
only  child  of  parents,  both  of  whom  also  suffered  from  mental 
disease.  Mrs.  M.'s  father  died  of  what  was  called  softening 
of  the  brain  (one  should  avoid  terming  all  old  cases  of  so-called 
11  softening  of  the  brain  "  syphilitic,  since  the  older  diag- 
nosticians did  not  always  distinguish  between  non-syphilitic 
arteriosclerotic  effects  and  syphilitic  disease).  Mrs.  M.'s 
mother  also  died  insane  (confusion  and  emotional  depression). 
It  is  clear,  then,  that  we  do  not  need  to  suppose  that  every 
symptom  shown  by  Mrs.  M.  is  directly  due  to  destructive  or 
irritative  lesions  immediately  due  to  the  spirocheta  pallida. 
The  case  is,  in  fact,  an  excellent  lesson  as  to  the  association  of 
structural  and  functional  effects  in  neuropathological  cases. 
Mrs.  M.  as  a  child  had  shown  talent,  but  was  somewhat 
nervous  and  eccentric.  At  one  time,  she  had  an  attack  of 
hysterical  dysphasia ;  at  another  time,  an  attack  of  hysterical 
dyspnea;  during  another  period,  an  apparent  obsession  (kick- 
ing the  mopboard  at  regular  intervals).  Moreover,  she  had 
for  years  suffered  from  migraine  of  a  severe  and  unusual  type. 


FORMS   OF  NEUROSYPHILIS  1 9 

Both  the  hysterical  tendency  and  the  migrainous  tendency 
became  mingled  with  the  results  of  the  neurosyphilis  in  later 
stages  of  the  disease  in  such  wise  that  it  was  hard  to  tell 
exactly  where  the  structural  phenomena  left  off  and  the 
functional  phenomena  began. 

For  example,  at  the  age  of  32,  nine  years  after  infection 
and  four  years  after  the  earliest  nerve  symptoms  traceable 
to  syphilis,  and  at  about  the  time  of  the  onset  of  spinal  cord 
symptoms,  an  attack  was  described  as  follows: 

The  patient  had  a  very  severe  attack  of  migraine  (?) 
yesterday,  preceded  and  accompanied  by  paraphasia, 
so  severe  that  for  three  hours  she  was  unable  to  make 
herself  understood,  and  indeed  felt  "as  if  her  ideas  were 
getting  away  from  her."  This  attack  was  ushered  in 
by  a  numbness  of  the  forefinger  and  thumb  of  the  right 
hand,  which  lasted  for  about  three  hours,  though  the 
earlier  attacks  had  lasted  for  only  about  ten  minutes. 
During  this  period  the  hand  felt  as  if  it  had  been  frozen 
and  the  loss  of  muscular  power  was  so  great  that  she 
was  unable  to  hold  objects  in  the  hand.  In  some  of 
the  attacks  this  paraesthesia  has  affected  the  entire  left 
half  of  the  body,  and  occasionally  the  right  half.  Some- 
times the  seizures  come  on  with  great  suddenness,  so  that 
once,  when  she  was  attacked  while  in  the  middle  of 
the  street,  she  had  considerable  difficulty  in  reaching 
the  sidewalk.  After  the  worst  part  of  the  attack  is 
over  a  certain  amount  of  paraphasia  may  persist  for 
some  days,  together  with  awkwardness  in  the  use  of 
the  right  hand  and  numbness.  She  has  had  a  great 
deal  of  nausea  and  vomiting,  without  reference  to  the 
taking  of  food.* 

Bearing  in  mind  the  mingling  of  structural  with  functional 
symptoms  in  this  case,  let  us  consider  the  autopsy  findings. 

Peripheral  neurosyphilis:  The  lesions  of  the  cranial 
nerves  were  characteristically  asymmetrical.  Whereas  the 
left  third  nerve  looked  entirely  normal,  the  right  third  nerve 
had  its  diameter  reduced  two-thirds.  On  the  other  hand, 
the  fourth  nerves  were  equal  and  apparently  normal.  The 
sensory  portion  of  the  left  fifth  nerve  was  normal ;  the  right 
fifth  nerve  was  normal.  The  right  sixth  nerve  agreed  with  the 

*  Notes  of  Dr.  James  J.  Putnam. 


20  FORMS  OF  NEUROSYPHILIS 


ANATOMICAL 

FORMS  OF  NEUROSYPHILIS 

AUTONOMIC  (SYMPATHETIC)  NEUROSYPHILIS? 
PERIPHERAL  NEUROSYPHILIS 

CENTRAL  NEUROSYPHILIS 
MENINGEAL 
VASCULAR 
PARENCHYMATOUS 
MENINGOVASCULAR 
VASCULOPARENCHYMATOUS 
DIFFUSE  ( =  MENINGOVASCULOPARENCHYMATOUS) 

GUMMA 


CHART  i 


FORMS   OF  NEUROSYPHILIS  21 


CLINICAL  FORMS   OF  NEUROSYPHILIS 

HEAD  AND  FEARNSIDES,  1914 

SYPHILIS  MENINGOVASCULARIS 
CEREBRAL  FORMS 
HEMIPLEGIA 

AFFECTION  OF  THE  CRANIAL  NERVES 
MUSCULAR  ATROPHY 

LATERAL  AND  COMBINED  DEGENERATIONS 
EPILEPSY 

SYPHILIS  CENTRALIS 

DEMENTIA  PARALYTICA 
TABES  DORSALIS 
MUSCULAR  ATROPHY 
OPTIC  ATROPHY 
GASTRIC  CRISES 
EPILEPTIC  MANIFESTATIONS 


CHART  2 


22  FORMS   OF  NEUROSYPHILIS 

right  third  nerve  in  being  atrophic,  and  was  in  fact  reduced 
to  a  mere  thread  without  contained  nerve  fibres  at  a  point 
2  mm.  from  its  superficial  origin.  Although  the  right  third 
nerve  was  atrophic,  it  was  the  left  seventh  and  eighth 
nerves  which  had  become  atrophic;  the  process  had  spared 
the  right  seventh  and  eighth  nerves.  The  remainder  of  the 
cranial  nerves  were  grossly  normal,  except  that  the  optic 
nerves  had  an  outer  zone  of  a  translucent  nature.  So  far, 
no  spirochetes  have  been  demonstrated  in  any  portion  of  the 
nervous  system  of  this  case,  but  such  asymmetrical  and  focal 
cranial  nerve  lesions  are  perhaps  due  to  local  spirochetal 
infection,  punctuating  (as  it  were)  the  diffuse  process. 

How  much  of  the  transient  blindness,  deafness,  and 
ocular  paralysis  can  be  explained  on  the  anatomical  findings 
in  these  nerves?  Possibly  a  portion  of  the  phenomena  can  be 
so  explained.  Thus,  the  mechanical  conditions  of  pressure 
inside  and  outside  these  nerves,  both  in  their  peripheral  course 
and  in  their  passage  through  the  membranes,  can  be  readily 
understood  to  differ  during  the  acute  and  subacute  inflam- 
mation, during  the  process  of  repair  in  the  pial  tissues,  and 
during  the  process  of  overgrowth  of  neuroglia  tissue  about 
the  superficial  origins  of  the  nerves.  Of  course,  the  majority 
of  lesions  of  these  nerves  were  entirely  extinct  at  the  time  of 
the  autopsy,  and  their  history  could  be  surmised  only  from 
the  appearances  in  the  left  eighth  nerve.  Here  occurred  a 
sharply  marked  focal  area  of  gliosis  with  apparently  total 
destruction  of  nerve  fibres  and  related  with  a  lymphocytosis 
of  the  investing  membrane  (one  of  the  few  areas  of  lymphocy- 
tosis found  anywhere  in  this  case). 

If  it  were  not  for  the  pre-infective  history,  the  hysterical 
dysphasia  and  dypsnea,  the  youthful  obsessions,  the  migrain- 
ous  tendency,  and  the  psychopathic  inheritance,  we  might  be 
tempted  to  try  to  explain  the  transient  blindness,  the  deafness, 
and  ocular  palsies  on  the  basis  of  mechanical  and  toxic  vari- 
ations in  the  conditions  of  the  peripheral  cranial  nerves. 
The  existence  of  a  trace  of  lymphocytosis  in  the  left  eighth 
nerve  leads  to  the  hypothesis  that  treatment  might  still  be 
effective  in  this  particular  region  (see  below  in  discussion  of 
spinal  symptoms). 


FORMS  OF  NEUROSYPHILIS  23 

Spinal  neurosyphilis :  Not  only  the  spinal  cord  but  also 
the  posterior  and  anterior  nerve  roots  exhibited  severe  lesions. 
These  lesions  were  both  meningeal  and  parenchymatous. 
The  meningeal  process  differed  in  its  intensity  in  different 
parts  of  the  spinal  cord,  being  severest  in  the  thoracic  region. 
At  one  point  in  this  region,  the  dura  mater  was  so  firmly 
attached  to  the  pia  mater  that  the  line  of  demarcation  be- 
tween the  two  membranes  was  hard  to  make  out.  In  fact, 
it  seems  clear  that  there  could  have  been  no  free  intercom- 
munication between  the  spinal  fluid  above  these  adhesions 
of  dura  to  pia  mater  and  the  spinal  fluid  below  the  adhesions. 
Accordingly,  it  seems  that  lumbar  puncture,  had  it  been 
practised  in  this  case,  would  have  failed  to  show  features  rep- 
resentative of  the  whole  cerebrospinal  fluid  system.  More- 
over, since  at  no  point  in  this  region  of  adhesions  or  in  the 
pia  mater  of  the  spinal  cord  below  this  point,  were  found  any 
lymphocytes,  it  seems  clear  that  the  ordinary  lumbar  punc- 
ture would  have  failed  to  reveal  a  pleocytosis.  Whether  this 
fluid  would  have  yielded  a  positive  globulin  and  excess  al- 
bumin test,  it  is  now  impossible  to  say;  but  it  appears  that 
the  process  in  the  lower  part  of  the  spinal  cord  was  to  all 
intents  and  purposes  extinct. 

However,  there  was  one  region  of  more  severe  inflammatory 
involvement.  The  spinal  cord  in  the  cervical  region  showed 
a  lymphocyte  infiltration  of  its  vessels  amounting  to  a  mild 
myelitis  (meaning,  thereby,  an  inflammatory  process  of  the 
spinal  cord  remote  from  the  pia  mater).  Moreover,  in  this 
region,  there  was,  besides  the  perivascular  infiltration  of  the 
substance,  also  an  infiltration  of  the  overlying  membranes 
themselves,  especially  in  and  near  the  posterior  root  zones. 

The  lessons  of  this  finding  are  several:  The  inflammatory 
process  in  this  case  does  not  appear  to  have  been  entirely 
extinct!  Can  we  not  suppose  that  treatment  might  still 
have  benefited  this  local  inflammation  (perivascular  infiltration 
of  the  cervical  spinal  cord  substance  and  overlying  lym- 
phocytic  meningitis)?  Can  we  not  also  picture  the  gradual 
ascent  of  the  inflammatory  lesions  from  lower  segments  to 
higher  segments  and  possibly  conceive  of  the  gradual  eleva- 
tion of  the  zone  of  hyperaesthesia  manifested  in  this  case  as 


24  FORMS  OF  NEUROSYPHILIS 

following  the  gradual  displacement  upward  of  the  lymphocytic 
process?  Are  there  spirochetes  in  this  tissue?  So  far  none 
have  been  discovered,  possibly  through  inaccuracies  of 
available  technique.  To  the  neuropathologist,  however,  the 
lesion  looks  like  a  local  reaction  to  organisms. 

In  addition  to  the  spinal  meningitis,  chronic  and  acute, 
as  above  described,  there  were  extensive  parenchymatous 
spinal  lesions. 

In  the  first  place,  the  meningitis  had  affected  practically 
all  the  posterior  roots  so  that  the  explanation  of  the  posterior 
column  sclerosis  of  this  case  is  clear.  The  meningitis  had 
apparently  been  so  marked,  also,  that  all  the  fibres  anywhere 
near  the  periphery  of  the  spinal  cord  had  been  likewise  de- 
stroyed. The  posterior  columns  and  the  posterior  root  zones 
were  markedly  sclerotic ;  or  as  we  say  (having  reference  to  the 
overgrowth  of  neuroglia  tissue)  gliotic.  But  there  was  as 
much  sclerosis  (gliosis)  of  the  lateral  columns  (particularly  in 
the  posterior  two-thirds)  as  there  was  in  the  posterior  columns 
and  root  zones.  In  fact,  the  entire  posterior  half  or  two-thirds 
of  the  spinal  cord  markedly  outstripped  the  anterior  portions 
of  the  cord  in  the  severity  of  the  gliosis  (sclerosis)  shown. 

But  although  we  can  explain  the  posterior  column  sclerosis, 
the  sclerosis  of  the  posterior  root  zones  and  the  marginal 
sclerosis  (Randsklerose)  round  the  entire  periphery  of  the 
cord,  on,  the  basis  of  long-standing  effects  of  old  meningitis, 
we  cannot  thus  explain  another  finding,  namely,  the  destruc- 
tion of  the  fibres  in  the  lateral  columns.  This,  in  fact, 
is  explained  through  lesions  (mentioned  below)  that  affected 
the  encephalon.  The  net  result  of  all  these  lesions  of  the 
spinal  cord  was  to  leave  only  the  gray  matter  and  a  small 
amount  of  surrounding  fibres  (belonging  to  short  tracts  uniting 
near-by  segments)  intact.  Briefly  stated,  every  long  tract  in 
the  spinal  cord  appeared  upon  examination  to  be  extensively 
degenerated.  The  genesis  of  this  parenchymatous  loss  was, 
however,  double,  being  in  part  due  to  a  local  meningeal  proc- 
ess (sometimes  known  as  "  perimeningitis  ")  and  in  part  due 
to  a  cutting  off  of  the  pyramidal  tract  fibres  on  both  sides  by 
lesions  higher  up  in  the  nervous  system. 

Can  we  offer  any  explanation  of  the  partial  return  of  knee- 


CASE  i.    SPINAL  CORD  (THREE  LEVELS)  SHOWING: 

A.  Marginal  sclerosis  —  effect  of  old  meningitis  now  extinct. 

B.  Posterior  column  sclerosis  —  effect  of  meningitis  about  posterior  roots  also  now  extinct. 

C.  Bilateral  pyramidal  tract  sclerosis  —  effect  of  cerebral  thrombotic  lesions. 

Note  distortion  of  tissues  in  B  and  C,  partly  artificial  (tissues  in  places  diffluent). 


FORMS  OF  NEUROSYPHILIS 


ANATOMICAL  FORMULAE 

MENINGOVASCULOPARENCHYMATOUS  INVOLVEMENT 

M,  V,  P,  or  Combinations  Applied  to  the  Classification 
of  Head  and  Fearnsides 


I.    SYPHILIS  MENINGOVASCULARIS 

CEREBRAL  FORMS  MorVorMV* 

HEMIPLEGIA  V 

AFFECTION  OF  THE  CRANIAL  NERVES  M 


MUSCULAR  ATROPHY 

LATERAL  AND  COMBINED  DEGENE- 
RATIONS 

EPILEPSY 

II.    SYPHILIS  CENTRALIS 

DEMENTIA  PARALYTICA 
TABES  DORSALIS 
MUSCULAR  ATROPHY 
OPTIC  ATROPHY 
GASTRIC  CRISES 

EPILEPTIC  MANIFESTATIONS 


M 

M 

MorV 


*  M  =  meningeal 
V  =  vascular 
P  =  parenchymatous 


CHART  3 


26  FORMS  OF  NEUROSYPHILIS 

jerks  after  their  temporary  total  loss  at  a  certain  period  of 
the  disease?  We  may  assume  that  the  knee-jerks  were 
functionally  lost  about  a  year  before  the  death  of  the  patient 
through  the  partial  or  even  almost  complete  destruction  of 
the  entering  posterior  root  fibres  at  that  level  of  the  spinal 
cord  which  is  directly  related  with  the  knee-jerk.  The  later 
partial  return  of  the  knee-jerks  apparently  requires  us  to 
suppose  the  maintenance  of  some  fibres  and  collaterals  by 
which  a  functional  connection  can  be  effected  between  the 
fibres  of  the  posterior  roots  and  the  anterior  horn  cells  which 
innervate  the  quadriceps  femoris.  Let  us  now  suppose 
that  pari  passu  with  the  actual  return  of  the  knee-jerks, 
the  destructive  processes  that  are  affecting  both  pyramidal 
tracts  high  up  in  the  nervous  system  are  now  advancing. 
It  is  clear  that,  whatever  inhibitory  influence  these  pyramidal 
tracts  have  been  exerting  up  to  this  time  upon  the  knee-jerk 
reflex  arc,  that  influence  is  now  to  be  decidedly  reduced  in 
amount  and  possibly  absolutely  lost.  Upon  the  loss  of 
such  inhibitory  influences  exerted  from  above,  the  few  per- 
sisting connections  of  the  posterior  roots  and  anterior  horn 
cells  are  now  permitted  to  resume  their  functions. 

Encephalic  neurosyphilis :  The  lesions  mentioned  above 
as  causing  destruction  of  the  pyramidal  tracts  of  the  spinal 
cord  were  symmetrically  destructive  and  atrophic  lesions  of 
the  gray  matter  of  both  corpora  striata  with  atrophy  of  the 
anterior  segments  of  the  internal  capsules.  There  was  a 
degenerative  process  of  the  corpus  callosum  especially  af- 
fecting the  forceps  minor  of  the  tapetum.  The  ventricles 
were  largely  dilated,  indicating  a  considerable  destruction  and 
atrophy  of  the  white  matter  in  general. 

After  the  above  discussion  of  the  possible  effects  of  py- 
ramidal tract  lesion  in  this  case,  it  is  unnecessary  further  to 
discuss  the  paraplegia  produced  by  the  cystic  lesions  of  the 
corpora  striata.  The  theorist  might  inquire  how  these 
cystic  lesions  are  produced :  whether  by  vascular  blocking  or 
by  toxic  effects  of  the  accumulations  of  spirochetes.  Evi- 
dence is  lacking  which  would  completely  sustain  either  hy- 
pothesis. Still,  we  do  know  that  lesions  almost  identical  in 
appearance  may  be  produced  by  the  necrosis  consequent  to 


FORMS   OF  NEUROSYPHILIS  2^ 

the  plugging  of  nutritive  vessels  in  an  organ  like  the  brain 
supplied  with  end  arteries.  Therefore,  it  is  probable  that 
most  pathologists  would  believe  these  lesions  of  the  corpora 
striata  to  be  produced  by  vascular  plugging  of  the  nature  of 
thrombosis. 

It  is  worth  while  to  note  that  there  was  a  suggestion  of 
foci  of  encephalitis  made  out  upon  the  gross  examination. 
The  cortex  in  general  showed  strikingly  few  lesions.  How- 
ever, the  convolutions  did  show  in  places  numerous  ill-defined 
areas  of  hyperemia  and  slight  swelling.  These  areas  were  of 
irregular  distribution  and  only  a  few  mm.  or  cm.  in  diameter. 
No  gross  vascular  lesions  were  demonstrable  in  connection 
with  these  focal  areas.  Microscopically,  however,  venous 
plugs  of  polymorphonuclear  leucocytes  were  found,  and  the 
local  hyperemias  were  found  to  be  largely  due  to  venous  con- 
gestion. However,  very  few  polymorphonuclear  leucocytes 
were  found  outside  the  blood  vessels. 

The  white  matter  of  numerous  convolutions  showed  mi- 
croscopically certain  pale  spots  suggestive  of  an  early  atrophic 
process.  Very  possibly  these  represent  a  general  tendency  in 
the  cerebrum  to  the  same  process  of  parenchymatous  loss  which 
had  proceeded  to  such  a  marked  degree  in  the  spinal  cord. 

There  was  a  single  large  so-called  cyst  of  softening  in  the 
cerebellum  (1.5  mm.  across  by  0.5-7.5  cm.  in  depth). 

How  far  can  we  explain  the  symptoms  of  this  case  on  the 
basis  of  these  encephalic  lesions?  We  can  offer  no  correlation 
with  the  cerebellar  lesion ;  and  possibly  this  lack  of  correlation 
is  to  be  expected  on  account  of  its  failure  to  affect  the  vermis. 
As  to  the  cystic  lesions  of  the  corpora  striata,  their  effect  in 
producing  paraplegia  at  the  close  of  life  is  obvious,  and  their 
possible  relation  to  the  partial  return  of  knee-jerks  has  been 
discussed.  Literally  amazing  was  the  comparative  integrity 
of  the  cortical  gray  matter  of  this  case  when  the  spinal  cord 
and  the  interior  structures  of  the  encephalon  had  been  sub- 
jected to  such  severe  and  numerous  lesions.  The  only 
mental  symptoms  noted  in  the  case  were  sundry  delusions 
directed  against  the  patient's  relatives  and  a  certain  optimism 
which  led  the  patient  to  cling  as  if  with  an  obsession  to  the 
belief  that  in  the  end  she  would  get  well. 


28  FORMS  OF  NEUROSYPHILIS 


VARIOUS  FORMS   OF  NEUROSYPHILIS 
COLLECTED  FROM  SEVERAL  SOURCES 

MENINGEAL  NEUROSYPHILIS  (M) 

GUMMA  OF  DURA  MATER  M 

GUMMATOUS  MENINGITIS  (?IAL)  M 

SYPHILITIC  MENINGITIS  (PIAL)  M 
SYPHILITIC  CRANIAL  NERVE  PALSIES  (PRIMARILY  PIAL)    M 

SYPHILITIC  BULBAR  PALSY  M 

SYPHILITIC  ROOT-NEURITIS  M 

SYPHILITIC  TRANSVERSE  MYELITIS  M 

SYPHILITIC  NEURITIS  (SOME  CASES  BY  EXTENSION)  M 

SYPHILITIC  EPILEPSY  (SOME  CASES)  M 

SYPHILITIC  MUSCULAR  ATROPHY  (SOME  CASES)  M 

VASCULAR  NEUROSYPfflLIS  (V) 

SYPHILITIC  ARTERIOSCLEROSIS  V 

SYPHILITIC  CEREBRAL  THROMBOSIS  V 

SYPHILITIC  APOPLEXY  V 

ANEURYSM  V 

SYPHILITIC  EPILEPSY  V 

PARENCHYMATOUS  NEUROSYPHILIS  (P) 

GUMMA  P 

CEREBROSPINAL  SCLEROSIS  P 

SYPHILITIC  PARANOIA  P? 

SYPHILITIC  CHOREA  P 

SYPHILITIC  EPILEPSY  P 

TABETIC  PSYCHOSIS  P? 

SYPHILITIC  MUSCULAR  ATROPHY  P 

SYPHILITIC  NEURITIS  P 

CHART  4A 


FORMS  OF  NEUROSYPHILIS  29 


MENINGOVASCULAR  NEUROSYPHILIS   (MV) 

CEREBRAL  SYPHILIS  MV 

CEREBROSPINAL  SYPHILIS  MV 

SYPHILITIC  EPILEPSY  MV 

MENINGOPARENCHYMATOUS  NEUROSYPHILIS  (MP) 

CEREBRAL  SYPHILIS  MP 

CEREBROSPINAL  SYPHILIS  MP 

TABES  DORSALIS  MP 

ERB'S  SYPHILITIC  SPASTIC  SPINAL  PALSY  MP 

VASCULOPARENCHYMATOUS  NEUROSYPHILIS  (VP) 

CEREBRAL  SYPHILIS  VP 

CEREBROSPINAL  SYPHILIS  VP 

PARETIC  NEUROSYPHILIS   (GENERAL  PARESIS)  VP 

LISSAUER'S  GENERAL  PARESIS  VP 

MENINGOVASCULOPARENCHYMATOUS   NEUROSYPHILIS    (MVP) 

CEREBRAL  SYPHILIS  MVP 

CEREBROSPINAL  SYPHILIS  MVP 

PARETIC  NEUROSYPHILIS  MVP 

TABOPARESIS  MVP 

DOUBTFUL  (TOXIC?,  IRRITATIVE  ?)  NEUROSYPHILIS  (?) 

"PARESIS  SINE  PARESI" 
SYPHILITIC  NEURASTHENIA 
TABETIC  PSYCHOSIS 
SYPHILITIC  PARANOIA 
SYPHILITIC  POLYURIA,  POLYDIPSIA 
SYPHILITIC  NEURALGIA 

CHART  4b 


3O  FORMS  OF  NEUROSYPHILIS 

Summary:  We  have  here  dealt  at  length  with  a  long- 
standing DIFFUSE  NEUROSYPHILIS  affecting  to  some  extent 
the  entire  meninges  and  producing  a  destruction  of  posterior 
column  fibres  and  numerous  other  fibres  of  the  spinal  cord 
(tabetifonn  portion  of  the  neurosyphilis  picture).  We  have 
also  found  central  lesions  of  the  corpora  striata  affecting  the 
destruction  of  both  pyramidal  tracts  (paraplegic  portion  of 
the  neurosyphilis  picture).  We  have  found  evidences  of 
acute  inflammation  (lymphocytosis)  in  the  cervical  region  of 
the  spinal  cord  and  in  the  left  eighth  nerve  (progressive 
inflammatory  neurosyphilis  picture).  In  short,  we  have 
presented  a  case  of  diffuse  (meningovasculoparenchymatous) 
neurosyphilis  characterized  by  an  ascending  character  in 
a  course  of  at  least  16  years;  we  have  indicated  a  number 
of  possible  clinical  correlations,  not  only  with  the  major  por- 
tion of  the  clinical  course  (symptoms  of  myelitis  and  pyramidal 
tract  destruction),  but  we  have  also  mentioned,  merely  for 
their  suggestive  value,  a  number  of  finer  correlations  be- 
tween histological  findings  and  certain  clinical  features  (no- 
tably transient  losses  of  vision  and  hearing,  and  a  partial 
return  of  the  lost  knee-jerks).  Bearing  in  mind  the  clinical 
and  anatomical  findings  of  this  case,  we  shall  be  able  to  discuss 
the  cases  that  follow  in  a  briefer  and  more  condensed  fashion. 


FORMS  OF  NEUROSYPHILIS  3! 


TABETIC  NEUROSYPHILIS  ("tabes  dorsalis," 
"  locomotor  ataxia ")  complicated  by  vascular 
neurosyphilis  (hemiplegia).  Autopsy. 


Case  2.  Francis  Garfield  had  been  a  successful  lumberman 
and  had  enjoyed  good  health  until  his  forty-fifth  year. 
Suddenly  one  day,  while  walking  on  the  street,  Garfield  lost 
the  use  of  his  legs  and  for  a  time  was  quite  unable  to  walk. 
However,  he  recovered  locomotion  and  after  a  time  there 
was  nothing  wrong  with  his  leg  movements  except  a  slight 
ataxia. 

At  the  age  of  52  Garfield  had  to  give  up  work.  It  appears 
that  he  had  been  becoming  cranky,  sometimes,  for  example, 
shouting,  whistling  and  slamming  doors,  apparently  to  annoy 
the  family.  His  intellectual  capacity  seemed  to  be  main- 
tained, although  his  memory  was  slightly  impaired. 

At  67  years  there  was  an  ill-defined  seizure,  followed  a  few 
days  later  by  another  seizure  with  aphasia  (wrong  words 
used  and  lack  of  understanding  of  things  said). 

For  years  Garfield  had  been  totally  deaf  in  the  right  ear 
(following  explosion  of  a  gun?).  Now,  however,  the  left 
ear  also  showed  a  sensory  impairment.  Slight  slurring  of 
speech  had  been  noticed  first  in  the  sixty-sixth  year. 

Physically  there  was  a  slightly  enlarged  heart  with  ac- 
centuated second  aortic  sound  and  irregular  rhythm.  Neuro- 
logically,  inability  to  stand  or  walk;  marked  ataxia  in  his 
leg  movements;  upper  extremities  quite  well  controlled; 
the  pupils  were  small  and  unequal,  the  left  being  larger  than 
the  right;  although  the  reactions  were  difficult  to  test,  the 
pupils  seemed  to  react  slightly  to  direct  light  stimuli;  the 
knee-jerks  were  absent;  tests  for  sensibility  so  far  as  could 
be  determined  did  not  show  any  abnormalities;  there  was 
much  complaint  of  sharp  pains  in  the  legs. 

There  is  no  doubt  that  we  are  here  dealing  with  a  case  of 
TABES  DORSALIS  plus  certain  complications  due  to  VASCULAR 
LESIONS.  The  case  went  on  to  death  from  rupture  of  aortic 


32  FORMS  OF  NEUROSYPHILIS 


MAIN  FORMS  OF  NEUROSYPHILIS 

(CLASSIFICATION  OF  THIS  BOOK) 


DIFFUSE  NEUROSYPHILIS 

(non-vascular  forms  of  "  cerebral,"  "  spinal "  and  "  cerebrospinal 
syphilis  ") 

VASCULAR  NEUROSYPHILIS 

("  cerebral  arteriosclerosis,"  "  cerebral  thrombosis  ") 

PARETIC  NEUROSYPHILIS 
("  general  paresis  ") 

TABETIC  NEUROSYPHILIS 
("  tabes  dorsalis  ") 

GUMMATOUS  NEUROSYPHILIS 
("  gumma  of  membranes,  of  brain  ") 

JUVENILE  NEUROSYPHILIS 
(paretic,  tabetic,  diffuse) 


CHART  5 


FORMS  OF  NEUROSYPHILIS  33 


POSSIBLE  INVOLVEMENT 

BRAIN  AND   CORD   SYPHILIS 
[M]embranes,  [Vjessels,  [Pjarenchyma 

[MVP]    EARLY,  LATENT  ?,  SYMBIOSIS  ?,  ATTENUATION  ? . . . . 

MVP       CEREBRAL,  CEREBROSPINAL  SYPHILIS,  PAR- 
ESIS MVP 

[M]VP    PARESIS;  SYPHILITIC  ARTERIOSCLEROSIS  VP 

M[V]P     ?  SYPHILOTOXIN  FROM   MENINGITIS  MP 

MV[PJ    SYPHILITIC     MENINGITIS;     CEREBRAL     OR 

CEREBROSPINAL  SYPHILIS  MV 

[MV]P    SYPHILOTOXIC  ATROPHY  OR  SCLEROSIS  P 

M[VP]    SYPHILITIC  MENINGITIS  M 

[M]V[P]    SYPHILITIC  ARTERIOSCLEROSIS  V 

M,  V  or  P  in  brackets  [  ]  means  not  involved. 


CHART  6 


FORMS  OF  NEUROSYPHILIS 


NEUROSYPHIL1S 

SIX  TESTS 


BLOOD  WASSERMANN 

SPINAL  FLUID  WASSERMANN 
"  "        CYTOLOGY 

"  "        GLOBULIN 

"  "       ALBUMIN 

"  "       GOLD  SOL 


CHART  7 


FORMS  OF  NEUROSYPHILIS  35 

aneurysm  (also  doubtless  a  syphilitic  complication).  The 
death  occurred  at  71,  four  years  after  admission  to  Danvers 
Hospital. 

This  case  has  been  especially  worked  up  and  published  by 
Dr.  A.  M.  Barrett  on  account  of  the  fact  that  the  vascular 
lesions  of  the  brain  had  produced  a  condition  of  pure  word- 
deafness.  Reference  is  made  to  the  Journal  of  Nervous  and 
Mental  Disease,  Vol.  37,  1910,  for  a  complete  description  of 
the  brain  findings  and  an  analysis  of  the  word-deafness,  a 
summary  of  which  is  as  follows : 

"  Reaction  to  Words  and  Sounds.  —  Total  deafness  to 
words  spoken,  but  gives  attention  to  sounds;  no  ability 
to  recognize  meaning  of  sounds  heard;  no  ability  to 
repeat  words  heard.  Spontaneous  Speech.  —  Retained 
ability  to  speak  spontaneously,  with  rare  paraphasic 
utterances;  occasional  inability  to  speak  readily  the 
word  desired,  but  later  always  giving  the  correct  reaction ; 
calculation  fair;  spelling  good  except  for  occasional 
paraphasia;  spelling  good  for  words  pronounced.  Re- 
action to  Things  Seen.  —  Objects  correctly  recognized 
and  named  except  for  an  occasional  paraphasic  reply; 
mistakes  in  pronunciation  not  recognized ;  correct  color 
recognition.  Reaction  to  Things  Felt.  —  Good  for 
familiar  objects;  an  occasional  paraphasic  reply.  Re- 
action to  Words  Seen.  —  Reads  printing  and  writing 
understandingly ;  unimpaired  reading  except  for  an 
occasional  paraphasic  reply;  meaning  of  familiar  signs 
recognized;  slight  difficulty  in  readily  understanding 
meaning  of  arithmetical  signs.  Writing.  —  Spontane- 
ous writing  and  drawing  ability  retained;  ataxia 
(tabetic)  in  writing  movements;  no  ability  to  write 
from  dictation.  Internal  language.  —  No  evidence  of 
impairment." 

The  brain  post  mortem  showed  severe  atheromatous  de- 
generation of  the  arteries  at  the  base  of  the  brain.  Both 
middle  cerebral  arteries  showed  scattered  atheromatous 
patches.  The  pia  mater  was  transparent  and  delicate,  except 
in  the  regions  of  both  Sylvian  fissures.  There  were  residuals 
of  old  softening  in  both  temporal  lobes.  In  the  fresh  brain 
the  regions  of  the  right  and  left  first  temporal  convolutions 
were  sunken  inward,  and  the  pia  intimately  adherent  to  the 


-6  FORMS  OF  NEUROSYPHILIS 

softened  areas.  ,  The  limits  and  more  exact  localizing  of  these 
softenings  were  worked  out  from  serial  sections. 

Barrett  found  in  his  serial  sections  that,  although  the  trans- 
verse temporal  convolutions  of  the  left  hemispheres  were  in- 
tact, these  convolutions  were  undermined  throughout  their 
entire  extent  by  degenerations  in  the  fibres  of  the  center  of 
the  first  temporal  convolution.  Barrett,  accordingly,  re- 
garded his  case  as  essentially  a  case  of  subcortical  tissue 
destruction.  He  agrees  with  various  authors  that  the  pure 
word-deafness  of  his  case  is  the  result  of  an  isolation  of  the 
receiving  station  in  the  transverse  convolutions  of  the  left 
hemisphere.  The  tissue  destruction  produced  by  the  vas- 
cular lesion  had  cut  off  the  transverse  convolutions  from 
the  internal  geniculate  body. 

We  are  here,  however,  not  considering  the  origin  and  rela- 
tions of  pure  word-deafness  but  present  the  case  as  one  of 
tabes  dorsalis  of  20  years  standing,  terminated  by  two  char- 
acteristic syphilitic  complications,  first,  an  extensive  destruc- 
tion of  brain  tissue  through  cerebral  thrombosis  and  secondly, 
fatal  aortic  aneurysm. 

Summary :  We  have  here  dealt  briefly  with  a  long-standing 
case  of  NEUROSYPHILIS  of  the  TABETIC  type:  A  characteristic 
but  not  necessary  complication  of  the  case  is  the  LATE  CERE- 
BRAL VASCULAR  INVOLVEMENT.  The  posterior  column  sclerosis 
is  virtually  the  only  spinal  change.  Spinal  meningeal  changes 
are  absent  (although  it  is  to  be  assumed  that  chronic  inflam- 
matory changes  in  the  posterior  roots  were  at  one  time  present 
in  some  quantity  and  although  the  spinal  fluid  characteristic- 
ally shows  lymphocytosis  in  tabetic  neurosyphilis) . 

Whether  the  spirochetes  produce  special  toxic  components 
able  to  cause  tabes  or  whether  special  kinds  of  spirochete  are 
the  tabes-making  kinds  is  hard  to  say.  Special  qualities  of 
individual  tissue  may  be  involved. 

The  cerebral  lesions  of  a  cystic  nature  are  of  vascular 
origin,  like  the  differently  localized  encephalic  lesions  of  Case 
I  (Alice  Morton).  Vascular  syphilis  is  not  a  special  property 
of  the  vessels  of  the  nervous  system.  In  fact  this  very  case 
died  of  aortic  aneurysm. 


FORMS   OF  NEUROSYPHILIS  37 


PARETTC  NEUROSYPHILIS  ("  general  paresis," 
"dementia  paralytica,"  "softening  of  the  brain"). 
Autopsy. 


Case  3.  James  Dixon,  44,  was  first  seen  at  the  Danvers 
Hospital,  reciting  verses  in  a  dramatic  and  noisy  way.  He 
remained  good-natured  and  jolly;  nor  was  there  any  change 
in  his  euphoria  until  he  had  become  physically  weaker  and 
more  generally  demented.  In  fact,  Dixon  appeared  to  be- 
come more  and  more  expansive  as  he  became  physically 
weaker.  He  was  in  the  habit  of  describing  himself  as 
"O.K.,  No.  I,  Superfine." 

Physically  the  patient  was  gray  and  bald  on  vertex,  had 
a  dusky  complexion,  was  very  thin  (6  ft.  in  height,  weight 
155  Ibs.);  the  mucous  membranes  were  pallid;  the  teeth 
rather  poorly  preserved;  the  heart  was  somewhat  enlarged; 
the  pulse  irregular  in  rhythm,  of  poor  volume  and  tension. 

Neurologically,  the  patient  showed  a  characteristic  Rom- 
berg  sign  and  ataxia  in  walking  a  straight  line.  The  tremulous 
tongue  was  protruded  to  the  left,  and  there  was  a  coarse 
tremor  of  the  extended  fingers.  The  knee-jerks  were  absent, 
and  the  Achilles  jerks  could  not  be  obtained;  the  plantar 
reactions  were  slight;  the  arm  reflexes  were  present.  The 
pupils  were  stiff  to  light.  There  was  a  marked  vocal  tremor. 
The  sensations  could  not  be  tested  on  account  of  the  patient's 
mental  state. 

It  appears  that  Dixon  had  left  school  at  about  16,  at  about 
22  had  gone  into  the  provision  business,  and  later  had  be- 
come a  hotel  clerk.  He  had  married  at  28;  there  had  been 
two  miscarriages,  at  three  months  and  six  weeks  respectively ; 
one  child  was  stillborn;  four  children  were  living. 

The  patient  was  not  very  alcoholic.  The  patient's  wife 
thought  the  symptoms  had  been  coming  on  since  his  forty- 
first  year  when  irritability  set  in,  but  he  was  not  discharged 
from  work  until  about  a  year  since.  He  was  taken  back  again 
after  his  wife's  pleas,  and  remained  at  work  about  three 


38  FORMS   OF  NEUROSYPHELIS 

months;  but  for  ten  months  before  admission  to  the  hospital, 
Dixon  had  done  practically  nothing,  had  shown  a  marked 
memory  failure  and  speech  defect,  at  the  same  time  claiming 
to  be  a  person  capable  of  doing  and  accomplishing  everything. 
He  had  become  careless  of  his  personal  appearance,  collected 
a  drawer-full  of  stumps  of  cigars,  carried  lumps  of  coal  in  his 
pocket,  laughed  causelessly,  and  spat  on  the  carpet. 

We  here  deal  with  a  case  of  unknown  duration  from  the 
initial  infection,  but  with  symptoms  lasting  about  three 
years  and  three  months.  Aside  from  the  cause  of  death 
(empyema  of  left  pleural  cavity  associated  with  acute  hemor- 
rhagic  splenitis,  acute  ileitis,  and  bronchial  lymphnoditis), 
the  body  showed  a  number  of  other  lesions  outside  the  nervous 
system.  There  was  the  usual  sclerosis  of  the  aorta,  though 
perhaps  less  marked  than  usual.  There  was  a  curious  acute 
arteritis  with  fusiform  dilatation  of  the  arteria  profunda 
femoris,  with  an  edema  of  the  thigh  muscles  and  blebs  of  the 
overlying  skin.  There  were  also  multiple  chronic  caseating 
lesions  of  the  liver,  without  evidence  of  fibrosis.  The  explana- 
tion of  these  liver  lesions  is  not  yet  clear.  There  was  a  cloudy 
swelling  of  the  kidney. 

The  calvarium  was  dense  and  the  dura  mater  thick  and 
adherent.  There  was  a  chronic  leptomeningitis,  which,  how- 
ever, was  rather  unusual  in  being  most  marked  in  the  posterior 
cisterna  and  along  the  sulci  of  the  cerebellar  hemispheres. 
There  was  a  general  cerebral  sclerosis,  with  a  question  of 
atrophy  of  the  superior  temporal  gyri  (suggesting  the  so-called 
Lissauer's  paresis).  There  was  a  marked  cerebellar  sclerosis 
with  a  consequent  sclerosis  (grossly  palpable)  of  the  commis- 
sural  fibres  of  the  pons.  There  was  a  generalized  slight  spinal 
sclerosis.  As  a  fair  sample  of  the  variety  of  head  findings  in 
paretic  neurosyphilis,  the  details  of  the  head  examination  are 
presented. 

^  Crown  bald,  with  a  slight  fuzzy  growth  of  short  hairs. 
Scalp  slightly  adherent  to  calvarium;  latter  of  usual 
thickness  but  denser  than  normal.  Dura  adherent 
to  calvanum  in  region  of  vertex;  dura  not  remarkable. 
Sinuses  normal.  Arachnoid  villi  moderately  developed. 
Pia  mater  a  trifle  thickened  and  rather  evenly  through- 


A.     Normal  postcentral  cortex. 
(Compare  B.) 


'""'  '•      •  -;jp. <*••*-:••'*  •' 

i^^m^^£IMd 


B.  Nerve-cell  losses.  Perivascular 
deposits  of  mononuclear  cells,  amongst 
which  are  numerous  plasma  cells.  Note 
decrease  in  number  of  nerve  cells.  Note 
irregular  disposition  of  nerve  cells. 
From  paretic  neurosyphilis. 


FORMS  OF  NEUROSYPHILIS  39 

out  the  cerebral  portion.  Linear  sulcal  markings  are 
remarkable  for  their  absence.  The  wall  of  the  cerebello- 
medullary  cisterna  is  thick  and  opaque.  The  most 
prominent  pial  thickenings  are  over  the  cerebellum. 
These  are  linear  or  may  show  feathery  out-growths  and 
are  seated  over  the  sulci,  particularly  in  the  neighbor- 
hood of  the  fissure  and  about  the  great  .cerebellar 
notch.  They  correspond  fairly  well  with  the  focal 
variation  in  consistence  of  under-lying  tissues  noted 
below. 

Brain  weight,  1265  grams.  Consistence  somewhat 
increased  throughout  and  somewhat  evenly  increased. 
The  prefrontal  region  shows  the  maximal  increase  of 
consistence  but  the  remainder  of  the  frontal  region  and 
corresponding  occipital  region  are  much  firmer  than 
normal.  The  two  superior  temporal  gyri  appear  to  be 
firmer  than  adjacent  gyri  and  are  possibly  slightly 
diminished  in  superficial  diameter.  The  hippocampal 
gyri  are  fairly  firm.  The  substance  on  section  is  a 
trifle  more  moist  than  normal.  The  gray  and  white 
matter  cut  quite  evenly.  Diminution  in  depth  of  gray 
matter,  if  existent,  could  not  be  demonstrated.  The 
ventricles  show  a  moderate  sanding  throughout,  best 
marked  in  the  fourth  ventricle.  The  basal  ganglia  are 
not  remarkable  except  for  the  development  of  numerous 
dilated  perivascular  spaces  about  the  lenticulostriate 
vessels.  The  pons  is  atrophic,  but  more  so  on  the 
right  side.  The  pons,  like  the  prefrontal  cortex,  shows 
on  section  a  distinct  increase  of  consistence  immedi- 
ately beneath  the  pia  mater.  The  white  bands  of  the 
pons  on  section  are  distinctly  firmer  than  the  interven- 
ing substance.  The  olives  are  of  equal  consistence. 
Weight  of  cerebellum,  pons,  and  medulla,  155  grams. 
The  cerebellum  shows  an  obvious  atrophic  and  gliotic 
process  of  a  symmetrical  character.  The  superior 
surface,  including  both  vermis  and  hemispheres,  shows 
a  consistence  above  normal  and  general  reduction  of 
the  depth  measured  from  the  white  matter.  The  re- 
duction in  depth  gives  rise  to  a  visible  depression  as 
compared  with  tissue  posterior  to  the  postclival  sulci. 
The  lobus  cacuminis,  though  slightly  raised  from  the 
surrounding  lobes,  is  equally  firm,  if  not  firmer.  The 
superior  and  inferior  surfaces  show  practically  an  equal 
increase  of  consistence.  The  dentate  nuclei  are  not 
especially  increased  in  consistence.  The  flocculi  are 
reduced  in  size  about  one-third. 


40  FORMS  OF  NEUROSYPHILIS 

There  was  slight  universal  increase  in  consistence  of 
Spinal  cord,  best  marked  in  lumbar  region. 

Microscopic  findings  are  here  presented  merely  in 
sufficient  detail  to  establish  the  diagnosis.  The  left 
superior  frontal  gyrus  shows  extensive  and  somewhat 
irregular  cellular  and  fibrillar  gliosis  of  the  plexiform 
layer,  together  with  an  increase  of  thickened  vessels 
having  lymphocytes  and  plasma  cells  in  their  sheaths. 

The  perivascular  infiltrations  are  most  extensive  in 
the  lower  layers  of  the  cortex.  The  lamination  is  in 
places  thoroughly  obscured,  except  that  representatives 
of  the  layer  of  large  external  pyramids  are  almost 
always  demonstrable. 

The  layer  of  medium-sized  pyramids  has  undergone 
more  numerical  loss  of  elements  than  have  the  other 
layers. 

Gliosis  of  white  matter. 

Specimens  from  the  cerebellum  show  a  destructive 
process  of  great  severity,  but  a  little  irregular  in  extent, 
affecting  chiefly  the  Purkinje  cell  belt.  The  Purkinje 
cells  are  often  absent  throughout  one  side  of  a  given 
lamina,  and  there  has  ensued  a  dense  accumulation  of 
neuroglia  cells  along  a  former  Purkinje  cell  belt,  to- 
gether with  a  considerable  gliosis  of  the  molecular  layer. 
Considerable  gliosis  of  the  white  matter,  both  diffuse 
and  perivascular  in  distribution. 

Perivascular  plasma  cell  infiltrations  as  in  cerebrum, 
but  largely  meningeal  or  in  the  white  matter. 

Sections  from  the  corpora  striata  demonstrate  a  mild 
and  early  granular  ependymitis,  considerable  subepen- 
dymal  gliosis  of  cellular  type,  considerable  perivascular 
gliosis  in  the  white  portions  of  the  tissue,  and  a  moder- 
ate infiltration  of  perivascular  sheaths  with  pigmented 
cells,  lymphocytes,  and  plasma  cells.  There  is  little 
evidence  of  alteration  in  the  nerve  cells.  Some  are 
unevenly  pigmented. 

Summary:  We  here  present  a  case  with  numerous  and 
widespread  neurosyphilitic  lesions.  However,  the  gross  cere- 
bral vascular  complications  of  Case  I  (Alice  Morton)  and  of 
Case  2  (Francis  Garfield)  are  notably  absent  in  James  Dixon. 
Rather  atypical  (there  seems  to  be  always  something  atypical 
in  cases  of  neurosyphilis  1}  are  the  liver  lesions  and  arteritis  of 
the  leg,  atypical,  that  is  to  say,  for  PARETIC  NEUROSYPHILIS. 


Apparent  new  formation  of  small  blood  vessel.     Photographed  by  Dr.  A.  M.  Barrett. 


Rod  cells  (Stabchenzellen)  in  paretic  neurosyphilis.     Photographed  by  Dr.  A.  M.  Barrett. 


Granular  ependymitis  —  microscopic  appearance  of  a  marked  example  of  "sanding" 
of  ventricle. 


FORMS   OF   NEUROSYPHILIS  4! 

Highly  typical  of  paretic  neurosyphilis  and  almost  constant 
therein  is  the  aortic  sclerosis. 

Characteristic  and  constant  in  paretic  neurosyphilis  is  the 
Plasmocytosis  and  Lymphocytosis,  Perivascular  in  distribu- 
tion about  small  cortical  vessels.  There  is  also  a  character- 
istic (though  characteristically  less  prominent)  Plasmocytosis 
and  Lymphocytosis,  Meningeal  in  distribution.  The  pleocy- 
tosis  of  the  spinal  fluid,  almost  constant  though  variable  in 
amount  in  life,  is  an  indicator  of  the  meningeal  picture  and 
less  directly  of  the  parenchymatous  picture. 

Granular  Ependymitis  ("  sanding "  of  ventricle  floors)  is 
characteristic  and  may  be  regarded  as  part  of  the  paren- 
chymatous picture.  This  ependymitis  is  an  indicator  how 
chemical  changes  could  be  readily  produced  at  least  in  the 
ventricular  fluids,  since  the  limiting  membranes  of  the  nerve 
tissue  are  here  subject  to  multiple  breaks.  The  "  sanding  " 
is  a  neuroglia  reaction  to  these  multiple  small  breaks  (Wei- 
gert's  explanation). 

Parenchymatous  losses  have  led  to  Atrophy  and  Sclerosis, 
of  very  varying  extent  in  different  parts  of  the  encephalon. 
The  atrophy  is  characteristic  in  paretic  neurosyphilis,  but  by 
no  means  constant.  Numerous  cases  have  come  to  autopsy 
without  clearly  denned  gross  atrophy.  Sclerosis  is  also  char- 
acteristic and  even  more  frequent  than  atrophy,  doubtless 
because  sclerosis  represents  an  earlier  phase  of  a  process 
eventuating  in  gross  atrophy. 

A  Tabetiform  Picture  characterizes  the  spinal  cord,  but 
in  this  case  the  tabetic  clinical  picture  did  not  precede  the 
paretic  clinical  picture.  We  are  consequently  to  regard  the 
tabetic  spinal  process  as  incidental  and  on  all  fours  with 
the  Cerebellar  and  Pontine  Atrophy, 


42  FORMS   OF  NEUROSYPHILIS 


VASCULAR  NEUROSYPHILIS  ("  syphilitic  cere- 
bral thrombosis").    Autopsy. 


Case  4.  James  Pierce  was  an  almshouse  transfer  to  the 
Danvers  Hospital  in  his  fiftieth  year.  He  died  three  years 
later.  The  accompanying  brain  pictures  demonstrate  so 
extensive  a  lesion  of  the  left  hemisphere  that  it  is  of  great 
interest  to  determine  if  possible  the  genesis  and  course  of  his 
disease.  It  appears  that  syphilis  had  been  acquired  some- 
where about  the  age  of  38  or  40,  so  that  the  total  duration  of 
the  process  was  between  13  and  15  years.  In  Pierce's  forty- 
third  or  forty-fourth  year,  he  had  a  shock  while  walking  in 
the  streets  of  his  native  city,  whereupon  he  was  subsequently 
transferred  to  the  Danvers  Hospital,  whose  data  have  been 
summed  up  as  follows/  (we  are  obliged  to  Dr.  Charles  T. 
Ryder  for  these  data) : 

Neurological  examination:  Neuromuscular  condition: 
Barely  able  to  walk  or  stand  without  assistance; 
hemiplegia  of  right  side;  swings  foot  out  and  drags  toe 
out  and  around  in  attempting  to  walk.  Right  hand 
held  by  side,  flexed  at  right  angle;  fingers  contracted 
and  thumb  thrown  across  palm.  Can  lift  arm  from 
side;  practically  no  movements  of  forearms  or  fingers; 
atrophy  of  deltoid,  arm,  forearm,  and  hand.  Mus- 
cular movements  of  left  upper  extremities  fairly  well 
performed;  good  strength. 

Cranial  nerves:  Refuses  to  respond  to  any  tests  to 
determine  hearing  or  vision,  but  evidently  hears  what  is 
said  to  him,  and  in  his  movements  gives  no  evidence  of 
deafness.  Right  corner  of  mouth  droops;  tongue  pro- 
trudes straight. 

Reflexes:  Pupils  dilated;  margins  irregular;  left 
pupil  larger;  they  vary  in  size  but  it  is  impossible  to 
determine  whether  the  variation  is  due  to  light  or  ac- 
commodation reflex.  Reflexes  of  right  side  extremely 
exaggerated  throughout;  there  is  little  ankle  clonus; 
babmski  is  not  obtained,  patient  holding  his  toes  in 
flexed  position  in  resisting  attempts  to  elicit  reflexes. 


FORMS  OF  NEUROSYPHILIS  43 

Sensations:  Reaction  to  pain  stimuli  on  either  side. 
Evidently  some  anesthesia  on  right  side,  but  pressure 
is  apparently  very  painful.  There  is  considerable 
spasticity  of  limbs  on  right  side  on  passive  motion. 
Too  demented  to  make  accurate  tests. 

The  above  examination  was  made  on  May  6,  1904. 
On  May  2Oth  the  record  states: 

There  is  almost  complete  sensory  aphasia  with  word 
deafness;  some  paraphasic  -circumlocution.  Many  of 
his  words  are  very  well  enunciated  but  have  no  meaning. 
Is  apparently  unable  to  recognize  objects  or  their  uses. 

Brother  stated  that  he  was  always  supposed  not  to  be 
over  bright.  Physician's  certificate  states  that  he  is 
epileptic,  averaging  two  attacks  per  week.  On  the 
1 5th  of  May  he  had  a  general  convulsion;  was  uncon- 
scious for  half  an  hour,  and  dull  and  drowsy  for  two 
hours  afterwards.  On  the  I9th,  he  had  a  similar  attack 
in  the  afternoon,  the  convulsion  lasting  a  minute,  and 
he  was  stuporous  for  an  hour. 

On  November  8th  he  had  a  severe  epileptic  convul- 
sion. His  body  was  curled  up  to  the  right.  The 
convulsive  seizure  lasted  for  two  minutes  and  was 
followed  by  complete  unconsciousness  for  an  hour, 
when  the  patient  roused  and  appeared  as  usual  in  a  few 
minutes.  From  that  time  to  December  I5th  he  had 
five  epileptic  convulsions;  he  was  much  more  feeble, 
and  unable  to  help  himself  as  much  as  formerly. 

Nov.  7,  1905:  Patient  has  had  occasional  convul- 
sions since  last  note,  but  none  during  the  last  three 
months.  He  is  confined  to  bed,  has  become  very  much 
demented,  and  shows  very  marked  speech  defect,  so 
that  he  is  almost  unintelligible.  He  understands  only 
the  simplest  directions.  Legs  are  considerably  con- 
tracted and  knees  are  flexed.  Arm  and  hand  on  the  right 
are  paralyzed  and  show  some  atrophic  changes;  par- 
tially flexed.  Left  elbow- jerk  is  very  lively.  On 
May  23,  1906  he  was  reported  as  having  Achilles  on 
right  side  only,  and  Babinski  on  right  side.  He  died 
January  5,  1907. 

The  autopsy  findings  were  as  follows: 

Head:  Calvarium  of  moderate  thickness;  diploe 
present;  dura  slightly  adherent  over  bregmatic  region. 
Longitudinal  sinus  contains  cruor  clot.  Dura  is  some- 
what thickened  and  slightly  more  opaque  than  normal. 
Pacchionian  granulations,  small  but  fairly  numerous. 
Pia  contains  throughout  a  considerable  excess  of  clear 


44  FORMS  OF  NEUROSYPHILIS 

serous  fluid.  The  convolutions  in  general  are  of  good 
breadth  and  proportion.  There  is  an  atrophic  area 
roughly  circular  in  outline  and  about  2  cm.  in  diameter 
in  the  posterior  part  of  the  right  third  frontal  con- 
volution corresponding  to  Broca's  area  on  the  opposite 
hemisphere.  The  space  thus  formed  is  filled  with  edema 
held  by  the  pia.  On  the  left  side  is  a  similar  subpial 
collection  which  covers  the  site  of  the  posterior  portions 
of  all  of  the  third  frontal  convolutions,  parts  of  the 
lower  end  of  the  precentral  convolution,  and  the  whole 
of  the  first  temporal  convolution,  which  have  disap- 
peared entirely.  The  basal  vessels  show  slight  changes. 

Cerebellum  and  basal  ganglia  are  grossly  normal. 

The  spinal  membranes  are  negative.  The  regions  of 
the  pyramidal  tracts  in  the  cord  are  firm,  project 
slightly  from  surface  of  section,  and  are  china  white. 

Summary:  Here  is  a  picture  made  up  almost  purely  of 
VASCULAR  NEUROSYPHILIS,  with  SECONDARY  SPINAL  (PY- 
RAMIDAL TRACT)  CHANGES.  Doubtless  the  genesis  of  this 
picture  is  allied  to  that  of  Case  I  (Alice  Morton)  and  to  that 
of  the  terminal  vascular  complications  in  a  tabetic,  Case  2 
(Francis  Garfield). 

The  absence  of  meningeal  and  parenchymatous  (i.e.,  out- 
side the  region  of  necrosis  produced  by  the  vascular  disease) 
lesions  is  characteristic  of  an  important  group  of  neuro- 
syphilitic  diseases.  It  is  clear  that  the  case,  although  one  of 
extensive  lesions,  is  not  one  of  diffuse  lesions  in  the  sense  of 
Case  i  (Alice  Morton). 

The  spinal  fluid  picture  in  life  may  nevertheless  show  (as 
other  cases  amply  demonstrate)  a  certain  amount  of  lympho- 
cytosis  and  possibly  plasmocytosis,  together  with  a  variety 
of  other  changes.  Treatment  might  be  expected  to  keep 
down  these  associated  changes,  although  obviously  the  effects 
of  the  necrosis  are  final  and  definite.  Franz  in  Washington 
has  succeeded  in  "reeducating"  some  of  these  hemiplegics, 
employing  lower  mechanisms  of  the  nervous  system. 


Case  4.     (See  previous  figure  for  brain  lesion.)     Three  levels  of  the  spinal  cord  showing 
unilateral  pyramidal  tract  sclerosis,  10  years  after  cerebral  thrombosis. 


FORMS  OF  NEUROSYPHILIS  45 


JUVENILE  PARETIC  NEUROSYPHILIS  ("  juve- 
nile paresis ").    Autopsy. 


Case  5.  John  Lawrence  was  an  under-sized  negro,  who 
came  under  hospital  observation  when  he  was  23  years  of 
age.  There  was  some  evidence  that  the  patient's  father  was 
a  neurosyphilitic  although  accurate  data  were  out  of  the 
question.  At  all  events,  John  had  Hutchinsonian  teeth,  a  for- 
ward bowing  of  the  tibiae,  and  Argyll-Robertson  pupils. 
These  findings  together  with  a  history  of  backwardness  at 
school  seem  to  stamp  the  diagnosis.  It  seems  that  there  had 
been  a  change  for  the  worse  from  the  age  of  18,  though  the 
boy  had  been  able  to  sell  newspapers  and  black  shoes  up  to 
within  a  year  of  his  arrival  at  the  hospital.  During  the  last 
months  of  his  life,  he  showed  a  general  incoordination,  with 
false  movements  suggesting  those  of  a  drunken  person.  There 
were  numerous  tremors,  the  glance  was  shifting,  and  there 
was  a  tendency  to  nystagmus.  Some  of  these  phenomena 
(taking  into  account  that  the  Hutchinsonian  teeth  were  not 
entirely  typical  and  there  was  even  at  times  some  doubt  as 
to  whether  the  pupils  were  actually  stiff)  led  to  a  question  of 
the  diagnosis  multiple  sclerosis. 

There  was,  however,  little  doubt  that  the  case  was  one  of 
juvenile  paresis.  Among  the  symptoms  found  at  various 
times  in  this  case  are  the  following:  disorientation  for  time, 
place  and  persons,  confusion,  with  coarsely  irrelevant  replies  to 
questions,  ill-defined  and  transitory  delusions  of  persecution, 
auditory,  tactile,  and  visual  hallucinations,  and  defective 
memory. 

Early  in  life,  the  patient  had  had  a  habit  of  falling  asleep 
in  school  hours,  and  had  experienced  a  number  of  falls  at 
various  times.  During  an  attack  of  measles  he  had  had  a 
number  of  spasms,  each  of  which  lasted  ten  minutes  or  more. 

The  autopsy  showed  death  to  be  due  to  an  early  bronchial 
pneumonia.  The  thymus  was  persistent,  measuring  3X2X 
.5  cm.  The  marrow  of  the  femur  was  red. 


46  FORMS  OF  NEUROSYPHILIS 

There  was  a  moderate  degree  of  sclerosis  of  the  aorta  con- 
fined to  a  few  plaques  in  the  arch  (not  a  characteristic  syphil- 
itic scarring  of  the  aorta).  The  spleen  was  small  and  had  a 
thickened  capsule. 

The  majority  of  the  lesions,  however,  were  in  the  nervous 
system,  and  the  following  description  is  taken  from  the  routine 
hospital  records  to  exemplify  the  findings  in  a  fairly  charac- 
teristic case  of  JUVENILE  PARESIS. 

Head:  Scalp  closely  adherent  to  calvarium.  Cal- 
varium  heavy  without  diploe.  Dura  adherent  to  cal- 
varium in  bregmatic  region.  Sinuses  contain  liquid 
blood.  Arachnoidal  villi  in  considerable  quantity.  Pia 
mater  contains  considerable  clear  fluid  and  shows  diffuse 
haziness  and  focal  thickenings.  The  diffuse  haziness 
is  almost  universal  and  is  best  marked  over  the  superior 
surface  of  the  cerebellum.  The  focal  thickenings  are 
of  general  distribution  over  the  veins  of  the  sulci  on  the 
superior  surface  of  the  brim  and  are  heaped  up  to  form 
considerable  linear  mounds  near  the  region  of  the  arach- 
noidal  villi.  The  superior  surface  of  the  cerebellum  is 
traversed  by  similar  linear  mounds  of  fibrous  tissue 
running  at  an  angle  to  the  laminae.  There  is  no  notable 
increase  of  fibrous  tissue  at  the  base. 

Brain :  Weight  965  grams.  The  sulcation  is  roughly 
symmetrical  except  in  the  occipital  poles  where  there  is 
unusually  rich  and  complex  but  shallow  sulcation.  The 
cortical  substance  is  everywhere  firmer  than  normal, 
but  the  sulci  fail  to  flare  notably.  In  a  few  places  there 
is  a  focal  increase  of  consistence  of  still  greater  degree 
with  apparent  local  hypertrophy  (or  gliosis  with  in- 
crease of  substance).  These  foci  are  in  the  right  second 
temporal  gyrus  (3  cm.  in  diameter)  and  in  the  left  first 
temporal  gyrus  (of  same  size  but  somewhat  less  firm) 
and  are  of  a  whitish,  waxen  appearance,  being  visible 
several  feet  away  by  reason  of  their  color  and  apparent 
encroachment  upon  the  adjacent  sulci.  The  foci  are 
sharply  limited  by  the  sulci  laterally,  but  pale  out 
gradually  before  and  behind. 

The  convolutions  of  the  vertex  show  another  type  of 
lesion.  The  tissue  of  the  greater  part  of  the  vertex 
resembles  that  of  the  flanks  and  base  in  being  firmer 
than  normal  and  of  a  grayish  pink  color.  Behind  the 
fissure  of  Rolando  on  the  right  side  and  behind  the  an- 
terior limits  of  the  ascending  frontal  region  on  the  left 


FORMS  OF  NEUROSYPHILIS  47 

side  the  brain  tissue  of  the  vertex  becomes  suddenly 
still  firmer  and  of  a  yellowish  gray  color.  This  lesion 
disappears  gradually  into  the  occipital  microgyria  be- 
hind and  the  gyri  gradually  lose  their  yellowish  tint. 
The  lesion  fades  away  gradually  so  that  it  fails  to  in- 
volve the  temporal  convolutions. 

The  cerebral  tissue  cuts  firmly  and  smoothly.  The 
tissue  of  the  frontal  region  is  a  little  edematous.  The 
white  matter  is  of  a  normal  appearance.  The  ependyma 
of  all  the  ventricles  is  somewhat  sanded.  The  fourth 
ventricle  is  most  affected. 

The  cerebellum  is  not  edematous  and  is  as  firm  as 
the  normal  olivary  bodies.  The  cerebellar  hemispheres 
are  symmetrical  and  of  a  normal  appearance,  save  that 
the  laminae  are  slightly  narrower  than  usual  and  very 
compactly  set.  The  color,  where  not  obscured  by  the 
haziness  of  the  pia  mater,  is  of  a  grayish  pink  somewhat 
suggestive  of  freshly  tanned  shoe  leather.  The  sub- 
stance cuts  smoothly  and  firmly.  The  dentate  nuclei  are 
unusually  firm.  The  pons  is  small,  but  of  the  usual 
color.  Lower  structures  normal  except  the  cord  which 
is  small  and  shows  curious  deviations  from  the  normal 
markings.  The  posterior  horns  and  gray  commissure 
are  at  many  levels  the  only  structures  to  preserve  the 
normal  gray  appearance,  so  that  the  H  or  butterfly 
appearance  is  replaced  by  a  crescent.  At  these  levels 
traces  of  gray  matter  often  stand  out  in  the  loci  of  the 
anterior  horns. 

The  important  anatomical  diagnoses  in  the  nervous  system 
are  as  follows: 

Atrophy  of  cerebrum,  965  grams  (there  is  of  course  a 
question  whether  we  are  not  dealing  with  a  degree  of  cerebral 
hypoplasia) . 

Focal  scleroses  of  cerebrum,  suggesting  the  tuberous 
scleroses  of  Bourneville. 

Occipital  microgyria. 

Cerebral  and  cerebellar  gliosis. 

Chronic  ependymitis. 

Gliosis  of  the  gray  matter  of  the  spinal  cord. 

Chronic  diffuse  and  focal  leptomeningitis. 

The  microscopic  examination  confirmed  the  diagnosis  of 
paresis.  The  hypertrophic  nodules  were  of  special  interest. 


48  FORMS  OF  NEUROSYPHILIS 

They  were  found  to  be  overlain  by  a  characteristic  though 
thin  exudate  of  lymphocytes  and  plasma  cells,  together  with 
pigmented  cells.  The  nodules  appeared  to  be  supplied  with 
an  unusual  number  of  vessels  of  small  calibre,  about  which 
were  a  few  lymphocytes.  The  large  vessels  and  those  with 
well-developed  adventitiae  were  surrounded  by  more  numerous 
lymphocytes  and  by  more  focal  accumulations  of  pigmented 
cells.  The  cortex  in  the  middle  of  a  nodule  had  almost  lost 
its  characteristic  cortical  layering.  The  cortex  was  here 
reduced  (specimen  from  temporal  lobe)  to  about  one-quarter 
of  its  normal  thickness,  and  was  found  to  be  composed  largely 
of  expanded  neuroglia  cells  and  vascular  tissue,  with  a  few 
nerve  elements,  small,  shrunken,  and  dark-staining.  The 
destructive  process  appeared  to  have  borne  hardest  on  the 
layer  of  internal  large  pyramids  and  the  fusiform  layer. 
There  was,  however,  nowhere  any  evidence  of  focal  necrosis 
such  as  ought  to  characterize  a  true  gumma.  The  sections 
stained  by  the  March!  method  failed  to  show  evidence  of 
fatty  degeneration  within  the  focus,  although  there  was  a 
marked  diffuse  accumulation  of  fatty  granulations  along  the 
nerve  fibres  in  the  underlying  white  matter.  A  special  study 
of  the  cerebellar  material  was  made  by  one  of  the  authors.* 
Occasional  Purkinje  cells  showed  the  characteristic  bi- 
nucleate  condition,  which  has  frequently  been  noted  in 
recent  literature. 

The  cerebellum  of  this  case  was  perhaps  the  most  markedly 
diseased  of  all  portions  of  the  nervous  system.  As  noted,  the 
cerebellar  tissue  was  exceedingly  firm.  How  far  the  notable 
incoordination  of  the  case  (he  was  observed  on  staff  rounds 
characteristically  curled  up  in  a  heap,  showing  quite  an  unusual 
degree  of  general  incoordination)  was  due  to  the  cerebellar 
lesions,  it  is  perhaps  not  possible  to  say. 

Summary:  John  Lawrence,  JUVENILE  PARETIC  NEURO- 
SYPHILIS, is  a  foil  to  Case  3  (James  Dixon),  paretic  neuro- 
syphilis  due  to  acquired  syphilis. 

Both  showed  Cerebral  Atrophy,  but  Lawrence  the  more 

*  E.  E.  Southard :  Lesions  of  the  granule  layer  of  the 
human  cerebellum;  Journal  oj  Medical  Research,  XVI,  1907. 


FORMS  OF  NEUROSYPHILIS  49 

markedly  because  of  hypoplasia  incidental  to  the  congenital 
origin  of  his  condition. 

Whereas  Dixon  gave  little  or  no  sign  of  stigmata,  Lawrence 
(besides  being  undersized,  having  suspicious  teeth,  and 
showing  at  autopsy  a  persistent  thymus)  showed  a  Hydro- 
myelia  and  curious  trefoil  shape  to  the  spinal  cord.  Dixon 
on  the  other  hand  had  liver  lesions  and  arterial  lesions  of  the 
leg. 

The  suggestion  of  Tuberous  Sclerosis  in  Lawrence  is  not 
found  in  Dixon ;  but  we  have  not  found  it  elsewhere.  Bour- 
neville  did  not  describe  tuberous  sclerosis  as  syphilitic. 

Binucleate  Purkinje  cells  emphasize  the  congenital  source 
of  the  lesions  in  Lawrence. 

Plasmocytosis  and  Lymphocytosis,  Perivascular,  and  (less 
marked)  Meningeal,  are  found  in  both  the  congenital  and 
the  acquired  cases,  as  also  parenchymatous  changes,  both 
nerve  cell  losses  and  gliosis.  Both  also  show  granular 
ependymitis. 

It  is  clear  that,  over  and  above  the  factors  of  destruction 
evident  in  both  Lawrence  and  Dixon,  the  congenital  case, 
Lawrence  exhibits  also  the  effects  of  arrest  (in  brief  not 
merely  atrophy  but  also  hypoplasia).  Early  treatment  is, 
therefore,  theoretically  indicated  in  the  juvenile  group,  which 
means  early  diagnosis.  Early  diagnosis  and  treatment  are 
still  more  to  be  recommended  because  these  juvenile  cases 
progress  often  very  slowly  at  first. 


FORMS  OF  NEUROSYPHILIS 


FOCAL  BASILAR  MENINGEAL  NEUROSYPH- 
ILIS ("  syphilitic  extraocular  palsy,"  plus  other 
symptoms).  Autopsy. 


Case  6.  Flora  Black,  a  housewife  of  43  years,  had  been 
tired  out  for  a  year  but  had  been  apparently  in  fair  health. 
She  awoke  one  day  with  double  vision  due  to  a  left  internal 
strabismus.  The  visual  difficulty  gradually  passed  away  so 
that  five  months  after  the  sudden  seizure  she  was  apparently 
quite  well  again.  There  was  one  exception:  about  three  or 
four  months  after  the  attack  of  diplopia,  Mrs.  Black  had  begun 
to  feel  a  kind  of  weakness  in  various  parts  of  the  face  and  there 
were  also  fairly  definite  paresthesise.  In  the  sixth  month 
after  the  initial  attack,  the  patient  began  to  be  unable  to 
chew  and  was  fain  to  support  the  lower  jaw  with  a  bandage 
to  aid  in  mastication.  Deglutition  was,  however,  quite  un- 
affected and]  there  was  never  any  regurgitation  of  food. 
There  were  pains  in  the  face,  the  forehead  and  the  back  of 
the  neck. 

Upon  physical  examination  at  entrance  to  a  general  hos- 
pital, no  changes  in  the  body  at  large  were  discoverable. 
There  was  a  slight  edema  of  the  ankles,  otherwise  no  sign 
of  bodily  disease. 

Conditions  in  the  head  were  as  follows:  The  facial  lines 
were  (notes  by  courtesy  of  Dr.  E.  W.  Taylor)  smoothed  out; 
both  upper  and  lower  eyelids  and  the  corners  of  the  mouth 
drooped  slightly  and  more  markedly  on  the  left  side.  There 
was  slight  photophobia  and  considerable  lachrymation. 
The  patient  was  unable  to  pucker  forehead,  nose  or  mouth. 
The  unsupported  lower  jaw  fell  and  the  patient  was  unable 
to  open  the  mouth  widely.  The  movements  of  the  tongue 
were  normally  performed.  Speech  was  mumbling.  Sen- 
sations of  touch,  heat  and  cold  were  preserved  all  over  the 
face  except  that  the  left  cheek  below  the  level  of  the  mouth 
yielded  a  less  accurate  registration  of  tactile  sensations. 
A  hot  test  tube  did  not  feel  as  hot  in  the  lower  left  cheek'as 


FORMS  OF  NEUROSYPHILIS  51 

elsewhere.  Quinine  and  sugar  could  not  be  tasted  over  the 
left  half  of  the  tongue  in  front.  Smell  and  hearing  were  also 
diminished  on  the  left  side.  It  appeared  that  there  was  a 
complete  paralysis  of  the  5th  and  7th  nerves  and  a  partial 
paralysis  of  the  8th,  nth  and  I2th,  as  well  as  a  defect  in 
smell. 

The  patient  died  suddenly,  three  weeks  after  admission, 
running  a  slight  temperature  during  her  stay.  The  autopsy 
showed  (rather  surprisingly)  a  double  ovarian  carcinoma 
with  metastases  into  the  retroperitoneal  glands.  Both 
kidneys  were  found  to  be  riddled  with  nodules  of  carcinoma. 
The  pelvic  veins  were  thrombosed  and  there  was  a  complete 
occlusion  of  the  pulmonary  artery.  There  was  a  riding  em- 
bolus  in  the  foramen  ovale  and  there  was  coronary  embolism. 

The  striking  nature  of  these  complications  and  the  interest 
of  the  case  neurologically  would  warrant  its  publication  in 
complete  detail.  We  here  present  the  case  with  utmost 
brevity  as  an  example  of  a  SYPHILITIC  CRANIAL  NEURITIS 
by  extension  from  the  meninges. 

The  brain  was  in  general  without  change  but  there  was 
a  considerable  exudate  over  the  entire  pontine  region  which 
had  involved  several  cranial  nerves.  The  5th  nerves,  es- 
pecially the  left,  showed  gross  effects  of  the  inflammatory 
lesion.  There  seems  to  be  little  or  no  doubt  that  this  neuritis 
was  of  syphilitic  origin  despite  the  complication  of  the  case 
with  carcinoma  of  the  ovary  and  despite  the  fact  that  the 
case  was  observed  and  came  to  autopsy  before  the  modern 
methods  of  systematic  diagnosis  could  be  applied.  It  is  the 
best  case  available  to  us  for  the  demonstration  of  a  focal 
cranial  nerve  lesion  of  the  type  characteristic  of  neurosyphilis. 
We  may  well  suppose  that  similar  conditions  would  have  been 
found  at  various  stages  in  the  development  of  Case  I  (Alice 
Morton).  The  pontine  region  of  Case  I  was  entirely  free 
from  lymphocy tic  exudate  at  the  time  of  the  autopsy.  Pos- 
sibly the  clearing  up  of  the  pontine  pia  mater  in  Case  I  was 
a  therapeutic  effect  of  the  thorough  treatment  therein  used. 
Whether  a  case  like  Mrs.  Black's  could  be  cured  (aside  from 
the  ovarian  carcinoma  and  its  complications)  by  the  insti- 
tution of  vigorous  systematic  treatment  is  a  matter  of  doubt. 


£2  FORMS  OF  NEUROSYPHILIS 

Still,  in  a  general  way,  these  cases  of  focal  syphilitic  neuritis 
are  among  the  most  favorable  cases  for  treatment. 

Summary:  We  present  the  case  of  Flora  Black  to  em- 
phasize how  slight  in  extent  and  theoretically  curable  neuro- 
syphilis  may  be.  We  fear  that  Case  I  (Alice  Morton)  may 
present  too  unrelieved  and  pessimistic  a  picture.  The 
extensive  vascular  lesions  and  complications  of  Alice  Morton, 
of  Case  2  (Francis  Garfield),  of  Case  4  (James  Pierce)  arrest 
attention  by  the  incurability  of  their  residual  effects  (if  we 
omit  modern  attempts  at  reeducation  of  lower  arcs).  On  the 
other  hand  the  unrelenting  progress  to  destruction  of  impor- 
tant parenchymatous  structures,  as  shown  in  the  paretic 
James  Dixon  (Case  3)  and  his  juvenile  replica  John  Lawrence 
(Case  5),  as  well  as  in  Alice  Morton  (Case  i)  and  the  tabetic 
Francis  Garfield  (Case  2),  lead  to  a  certain  justifiable  pessi- 
mism. For  it  is  only  the  meningeal  and  fine  vascular  in- 
filtrations of  these  cases  that  we  can  theoretically  hope  to 
combat,  probably  by  destroying  the  spirochetes  in  these 
meningeal  and  perivascular  loci.  We  seem  theoretically  less 
able  to  stop  the  progress  of  the  often  highly  systemic  and 
symmetrical,  parenchymatous  lesions  of  the  tabetic  and 
paretic  group. 

The  condition  in  Flora  Black  is  clearly  much  more  hopeful, 
both  being  more  focal  and  being  almost  purely  meningeal 
and  therefore  accessible  to  therapy. 

The  two  cases  which  conclude  our  general  survey  of  neuro- 
syphilis  are  also  focal  cases,  one  of  gumma  (Lecompte)  and 
one  of  focal  dural  lesion  (Wyman). 


i.     Pons,  normal  except  for  focal  infil- 
tration of  left  fifth  nerve. 


2.    Higher  power  view  of  infiltrated  left 
fifth  nerve. 


*  "«    •  ^»» 


3.  Detail  of  infiltrated  left  fifth  nerve, 
showing:  i,  diffuse  infiltration  with  mononu- 
clear  cells;  2,  perivascular  infiltration;  3, 
strands  of  relatively  unaffected  nerve  fibers. 

Microscopic  appearances  in  Case  6.  Extraocular  palsy  (focal  meningeal  syphilis,  especially 
of  left  fifth  nerve).  Illustrates  exquisite  focality  of  the  syphilitic  process  sometimes  found, 
as  well  as  its  unilaterality  (giving  rise  to  asymmetrical  symptoms  and  signs).  Process  in 
itself  probably  curable. 


FORMS  OF  NEUROSYPHILIS  53 


GUMMATOUS  NEUROSYPHILIS   ("  gumma  of 
brain ").    Autopsy. 


Case  7.  Mrs.  Lecompte  was  a  woman  of  middle  age,  who, 
according  to  the  history  given  by  her  son,  had  been  entirely 
well  until  her  final  illness,  which  began  approximately  two 
years  before  admission  to  Danvers  Hospital.  The  beginning 
of  her  trouble  seemed  to  be  chiefly  headaches,  which  would 
last  continuously  for  several  days,  or  more  than  a  week  at  a 
time.  These  headaches  lasted  throughout  the  course  of  the 
disease.  In  the  morning,  on  arising,  she  would  feel  very  dizzy, 
but  this  would  pass  away  during  the  day.  She  had  had  a 
number  of  spells  of  unconsciousness,  lasting  about  fifteen 
minutes.  In  these  attacks  she  would  breathe  heavily,  there 
was  frothing  at  the  mouth,  twitching  of  the  hands,  and  the 
eyes  would  roll  about.  Her  memory  failed  gradually,  her  dis- 
position changed  and  she  became  very  irritable.  Vomiting 
occurred  almost  every  day,  and  at  times  was  of  a  projectile 
character.  She  became  hallucinated ;  the  hallucinations  were 
chiefly  of  a  visual  nature. 

About  four  months  before  admission  to  the  hospital,  after 
one  of  her  seizures,  the  entire  right  side  was  found  to  be  com- 
pletely paralyzed,  and  she  complained  that  it  was  numb. 
At  this  time,  she  had  difficulty  with  her  speech.  In  a  few 
days,  however,  she  was  able  to  talk  correctly  again,  and  in  a 
week  she  was  back  at  work,  although  the  right  side  was  weak 
and  awkward.  She  continued  to  grow  worse,  and  then  be- 
gan to  have  spells  lasting  several  days,  so  that  it  became 
necessary  to  have  her  placed  in  a  hospital. 

On  admission  to  the  hospital,  aside  from  obesity,  the  general 
viscera  showed  no  points  of  special  interest,  and  there  was 
no  evidence  of  any  new  growth  outside  of  the  nervous  system. 
She  was  unsteady  on  her  feet,  standing  with  them  wide  apart. 
The  gait  was  quite  ataxic;  the  whole  right  side  was  weaker 
than  the  left  and  used  more  awkwardly.  There  was  a  paraly- 
sis of  the  right  side  of  the  face;  the  right  angle  of  the  mouth 


CA  FORMS  OF  NEUROSYPHILIS 

drooped;  the  right  eyelid  could  not  be  closed  but  remained 
continuously  open;  nor  could  the  right  side  of  the  forehead 
be  wrinkled.  Vision  and  hearing  were  not  affected.  She 
miscalled  tastes  and  smells;  whether  this  was  due  to  aphasic 
difficulties  or  to  cranial  nerve  involvement  could  not  be 
divined.  There  seemed  to  be  some  difficulty  in  deglutition. 
The  knee-jerks  were  markedly  exaggerated;  slight  clonus 
was  obtained  but  was  not  always  present.  Both  pupils 
reacted  well  to  light  and  distance  and  consensually.  Sen- 
sation could  not  be  readily  tested.  There  was  marked  ataxia, 
especially  with  the  eyes  closed.  The  speech  was  thick  and 
mumbling.  The  patient  was  unable  to  write  or  copy.  Men- 
tally the  patient  was  quite  dull;  at  times,  stuporous;  when 
aroused,  was  found  to  be  entirely  disoriented.  Memory  al- 
most entirely  absent.  In  general  she  showed  herself  to  be 
very  much  confused. 

She  remained  practically  in  this  condition,  even  gaining  in 
weight,  for  the  following  two  years,  when  suddenly  one 
morning,  she  had  an  epileptic  seizure,  vomited,  coughed  a 
great  deal,  with  bleeding  from  the  mouth  and  ears,  and  died 
in  a  few  hours. 

The  symptoms  in  this  case  pointed  to  brain  tumor.  The 
only  inconsistent  thing  was  the  long-continued  life,  —  four 
years,  —  after  the  symptoms  were  observed.  As  she  lived 
before  the  W.  R.  and  spinal  fluid  tests  were  known,  no  light 
was  gained  in  these  ways.  The  post-mortem  examination 
showed  the  patient  had  a  GUMMA  OF  THE  BRAIN. 
The  summary  of  the  anatomical  diagnoses  at  autopsy  was: 

Decubitus. 

Lymphadenitis  of  the  mesenteric  nodes. 

Chronic  fibrous  peritonitis. 

Chronic  fibrous  myocarditis. 

Pulmonary  hypostasis. 

Thrombosis  of  vein  in  right  adrenal,  with  hemorrhage. 

Syphilitic  leptomeningitis. 

Gumma  of  left  hemisphere. 

Focal  softenings  in  the  pons. 

The  anatomical  description  of  the  head  (Dr.  A.  M.  Barrett) 
is  as  follows: 


FORMS   OF   NEUROSYPHILIS  55 

The  sutures  in  the  calvarium  are  well  outlined; 
diploe  large  in  amount.  The  dura  is  diffusely  but 
lightly  adherent  to  the  calvarium;  it  is  very  dense, 
especially  over  the  left  hemisphere..  The  meningeal 
arteries  are  thickened  but  not  atheromatous.  The 
sinuses  contain  a  small  amount  of  fluid  blood  and  post- 
mortem clot.  The  inner  surface  shows  nothing  abnor- 
mal. There  is  a  great  flattening  of  the  convolutions  of 
the  left  hemisphere,  which  is  not  the  case  on  the  right 
side.  Over  the  convexity,  the  pia  is  thin  and  not  ab- 
normal except  for  some  slight  adhesions  between  the 
frontal  lobes  and  the  two  lips  of  the  Sylvian  fissures. 
The  pia  at  the  base  over  the  cisterna,  pons,  and  medulla 
is  thick,  cloudy,  and  of  a  grayish  gelatinous  appearance. 
It  is  so  thick  that  it  is  easily  removable  in  a  large  piece. 

The  surface  of  the  left  hemisphere  is  dry,  and  the 
whole  brain  is  flabby  and  bulges  as  if  from  internal 
pressure.  A  section  through  the  hemispheres  at  the 
region  of  the  optic  chiasm  shows  a  hard,  firm  area  in  the 
left  hemisphere  deep  down  in  the  white  substance.  It 
is  about  2\  cm.  in  diameter,  with  a  wavy  border.  The 
central  part  is  of  a  silver-gray  gelatinous-like  appear- 
ance, with  red  spots  and  whitish  streaks  radiating  from 
the  centre.  In  the  pons  on  the  right  side,  in  a  plane  pass- 
ing through  the  posterior  corpora  quadrigemina,  are  two 
pinhead  size  softenings  among  the  pyramidal  fibres. 
The  ependyma  of  the  fourth  ventricle  is  granular. 

Microscopic  examination  of  the  tumor:  The  area 
evidently  contains  several  central  necrotic  foci  sur- 
rounded by  zones  of  infiltration  and  proliferation,  with 
bordering  areas  of  nervous  tissue  showing  secondary 
reactions.  The  necrotic  area  stains  poorly.  From 
the  edge  there  are  projections  of  reddish  homogeneous 
bands,  some  intermixed  with  well-differentiated  fibril- 
Ise,  probably  glia  fibrils.  The  bordering  zone  is  densely 
infiltrated  with  lymphoid,  plasma,  and  a  few  epithelial 
cells.  The  nerve  tissue  outside  of  this  zone  is  spongy 
and  infiltrated  with  lymphoid  and  plasma  cells.  There 
are  a  few  scattered,  shrunken  nerve  cells.  In  this  zone 
and  in  the  zone  of  infiltration  near  the  necrotic  area, 
there  are  scattered  cells  resembling  giant  cells.  There 
are  many  obliterated  vessels  in  the  area,  and  other  ves- 
sels show  many  infiltrating  lymphoid  and  plasma  cells 
in  the  walls.  The  examination  of  the  specimen  stained 
by  the  methods  for  bacilli  of  tuberculosis,  are  negative. 
The  growth  is  a  classical  gumma. 


FORMS  OF  NEUROSYPHILIS 


GUMMATOUS  NEUROSYPHILIS  (gumma  of 
spinal  meninges,  "  meningitis  hypertrophica  cervi- 
caHs  of  Charcot?")-  Autopsy. 


Case  8.  John  Wyman  was  first  seen  in  his  thirty-sixth 
year  by  Dr.  James  J.  Putnam.  He  denied  syphilitic  infection 
and  stated  that  the  first  symptoms  had  come  four  months 
before.  He  had  begun  to  notice  a  numbness  of  the  fingers,  at 
first  of  the  right  hand  and  shortly  thereafter  of  the  left  hand. 
After  a  few  weeks  there  had  been  difficulty  in  walking,  and  a 
few  weeks  later  headaches,  especially  on  the  right  side,  devel- 
oped. Two  weeks  before  he  was  first  seen  medically,  he 
had  begun  to  have  a  feeling  of  tightness  or  constriction  in 
his  arms. 

It  appears  that  micturition  had  been  impaired  early, 
that  is  to  say,  a  few  weeks  after  the  initial  sensory  disorder 
had  begun.  A  catheter  was  used  for  a  time  and  improvement 
followed.  Shortly  before  consultation  retention  of  urine 
developed  again,  this  time  associated  with  rectal  incon- 
tinence. The  feet  began  to  feel  heavy  and  dead.  Then 
the  legs  began  to  be  increasingly  weak  so  that  the  patient 
was  almost  bed-ridden.  Vision  appeared  to  be  normal  ex- 
cept that  reading  was  followed  by  fatigue.  The  speech 
was  also  slow  but  the  slowness  could  be  attributed  to  fatigue. 

Notes  of  Dr.  Putnam's  physical  examination  are  as  follows : 
The  patient  lay  in  bed  on  the  left  side,  without  motion,  and 
almost  incapable  of  motion.  The  tongue  was  protruded, 
and  there  was  no  paralysis  of  facial  muscles,  or  of  the  eye 
muscles  (the  right  pupil  had  been  reported  to  be  slightly 
larger  than  the  left).  There  seemed  to  be  a  disinclination  to 
move  the  head,  but  with  some  effort  it  could  be  moved,  and 
without  pain.  The  arms  and  hands  were  held  rigidly  in 
median  positions;  many  movements  were  possible,  but  all 
were  imperfect  and  of  slight  amplitude.  The  fingers  were 
flexed  to  a  moderate  degree,  and  could  not  easily  be  straight- 
ened, and  there  was,  in  fact,  a  general  rigidity  of  most  of  the 


FORMS   OF   NEUROSYPHILIS  57 

muscles  of  the  body  below  the  neck,  and  even,  in  some  degree, 
of  the  neck.  The  immobility  was  so  great  that  the  general 
impression  made  was  almost  that  of  a  patient  with  fracture  of 
the  spine  in  the  cervical  region.  Even  the  breath,  and  es- 
pecially the  inspiration,  was  imperfect.  The  legs  were  more 
freely  movable  than  the  arms,  but  still  the  motions  were  very 
stiff  and  awkward,  and  of  slight  amplitude;  with  effort  the 
whole  leg  could  be  lifted  from  the  bed,  and  flexed  or  extended 
with  moderate  force.  The  right  leg  was  rather  stronger  than 
the  left,  but  the  left  hand  and  arm  were  stronger  than  the 
right.  The  sensibility  was  almost  absent  over  the  hands 
and  lower  part  of  the  arms,  and  was  impaired  over  the  entire 
head  and  neck,  except  the  forehead,  the  middle  part  of  the 
face,  and  the  nose.  It  is  interesting  to  compare  the  con- 
ditions of  the  sensibility  here  present  with  those  seen  in 
cervical  syringomyelia.  The  sensibility  of  the  upper  part 
of  the  forehead  was  less  good  than  of  the  lower  part,  and  there 
was  slight  impairment  even  over  portions  of  the  lower  jaw. 
The  sensibility  of  the  left  (stronger)  arm  was  rather  more  im- 
paired than  that  of  the  right  arm,  while  on  the  contrary  the 
sensibility  of  the  left  leg  was  better  than  that  of  the  right 
leg,  though  the  difference  between  them  was  not  great.  These 
statements  apply  to  sensory  tests  by  contact,  heat,  cold,  and 
pricking.  Knee-jerks  were  highly  exaggerated,  and  likewise 
the  wrist- jerks.  All  forced  attempts  at  movements  were  at- 
tended by  a  high  degree  of  muscular  tremor,  especially  when 
the  patient  was  fatigued  or  under  emotional  strain.  The 
fingers  especially  were  the  seat  of  coarse  tremor. 

The  remainder  of  this  clinical  description  (courteously  sup- 
plied us  by  Dr.  Putnam)  may  be  quoted.  A  second  ex- 
amination which  included  also  a  few  facts  not  given  in  the 
first  examination  was  made  on  the  following  March  28,  1905. 
This  report  says  "  the  ends  of  the  fingers  became  numb  about 
June  I,  1904.  Work  was  given  up  on  July  3,  and  at  that 
time  the  patient  was  walking  very  badly.  No  treatment  was 
used  and  no  satisfactory  diagnosis  made.  In  the  course  of 
July  he  improved  somewhat,  and  during  August  he  was  able 
to  ride  out  a  little  (these  spontaneous  improvements  are  of 
interest  for  the  diagnosis).  He  went  away  from  home  for 


5g  FORMS  OF  NEUROSYPHILIS 

a  short  time,  but  from  the  time  of  his  return,  about  the  last' 
of  September,  he  grew  worse  rapidly,  and  fell  into  the  con- 
dition above  described,  in  which  he  was  wholly  unable  to 
help  himself,  even  to  turning  in  bed.  At  times  he  had  a 
great  deal  of  pain  in  the  neck  and  forehead.  Antisyphilitic 
treatment  was  recommended,  and  for  a  time  potassium  iodid 
and  other  iodid  preparations  were  given,  but  at  first  in 
relatively  small  doses  (grs.  75  daily).  Under  this  treatment 
the  excretion  of  urine  rose  to  four  quarts  daily  as  a  maximum 
though  sometimes  the  quantity  was  not  so  great." 

Under  this  treatment  the  patient  began  soon  to  improve, 
and  continued  doing  somewhat  better  till  about  five  months 
later.  He  became  able  to  walk  downstairs  and  out  of  doors, 
and  regained  considerable  use  of  his  hands.  The  quantity 
of  urine  passed  became  greatly  increased  by  the  use  of  the 
iodid. 

About  the  middle  of  March  he  became  worse  again.  A 
careful  examination  of  the  sensibility  showed  that  in  general 
the  condition  was  much  the  same  as  that  previously  reported. 
The  iodid  treatment,  with  perhaps  some  mercurial,  was 
resumed;  the  potassium  iodid  was  given  in  doses  which  were 
increased  up  to  850  grains  daily,  although  this  maximum  dose 
was  taken  only  for  about  one  week.  This  large  quantity 
gradually  impaired  the  sense  of  taste  for  the  time  being,  and 
blurred  his  vision,  but  otherwise  did  him  no  harm.  Under 
this  he  improved,  so  that  he  became  able  to  run  more  or 
less,  and  went  about  freely,  and  attended  to  his  business, 
though  still  retaining  some  stiffness  in  his  movements. 

This  improvement  continued  until  about  two  years  later, 
when  he  again  had  a  relapse,  and  was  seen  medically  once 
more.  His  condition  at  this  time  was  still  a  pretty  good  one, 
but  the  movements  were  stiff  and  awkward.  The  bin-iodid 
of  mercury  was  advised,  which  was  taken  in  doses  of  -fa  grain 
daily.  It  will  be  remembered  that  this  was  long  before  the 
days  of  salvarsan  treatment. 

This  was  toward  the  end  of  June,  1907.  Contrary  to  ex- 
pectation, there  was  no  material  gain  from  this  treatment, 
and  the  patient  died  early  in  October,  without  being  seen 
again. 


FORMS  OF  NEUROSYPHILIS  59 

t  The  autopsy  was  limited  to  the  nervous  system  and  the 
findings  were  as  follows  (Dr.  A.  R.  Robertson) : 

Head:  Hair  abundant,  fair,  of  fine  texture  and 
rather  curly.  Scalp  of  medium  thickness  and  strips 
readily  from  calvarium.  The  latter  appears  normal 
and  upon  removal  is  of  about  the  normal  thickness. 
It  lifts  readily  from  the  dura  mater,  except  for  the 
numerous  attachments  of  Pacchionian  granulations. 

Meninges :  The  dura  is  smooth,  moderately  injected 
and  shows  no  areas  of  thickening;  it  lifts  readily  from 
the  pia-arachnoid.  The  pia-arachnoid  shows  discrete 
and  in  many  places  diffuse  areas  of  opacity.  There  is  a 
moderate  amount  of  subpial  clear  fluid  and  the  vessels 
are  moderately  injected.  Over  the  anterior  surface 
of  the  medulla  and  lower  portion  of  the  pons  and  largely 
confined  to  the  right  side  there  is  a  very  marked  thick- 
ening of  the  pia-arachnoid  to  which  the  dura  is  densely 
adherent.  This  thickening  extends  down  anteriorly 
and  laterally  on  the  right  side  over  the  upper  part  of  the 
cervical  cord.  The  thickened  meninges  over  the  upper 
part  of  the  medulla  completely  surround  the  right 
vertebral  artery,  shortly  before  it  joins  its  fellow  of  the 
opposite  side  to  form  the  basilar.  Dissection  of  the 
arteries  shows  them  to  be  patent  and  thin  walled. 
Over  the  cerebrum  and  cerebellum  the  pia-arachnoid 
strips  readily  leaving  a  smooth  surface.  Section  of 
the  cerebral  cortex,  basal  nuclei,  pons  and  cerebellum 
show  no  gross  lesions.  The  ventricles  are  moderately 
distended  with  fluid.  The  ependyma  contains  nu- 
merous small  cysts.  Section  of  the  pons  shows  no 
lesions  of  the  nervous  tissue,  but  very  marked  thicken- 
ing of  the  surrounding  meninges  as  noted  above. 

Cord:  Throughout  the  cervical  and  dorsal  region 
the  dura  is  quite  tensely  distended  with  an  abundance 
of  clear,  light,  straw-colored  fluid.  Upon  snipping  the 
dura  this  fluid  escapes  with  a  small  spurt,  as  if  under 
considerable  pressure.  The  cord  within,  for  the  most 
part,  lies  free,  but  over  the  upper  three  or  four  centi- 
meters of  the  cervical  portion  it  is  densely  adherent  to 
the  dura  anteriorly  and  laterally  on  the  right  side. 
Cross  sections  were  made  through  the  upper  three  or 
four  centimeters  of  the  cord,  and  over  this  area  the  cord 
is  constricted  by  very  marked  thickening  of  all  the 
meninges.  The  meninges  here  average  from  one  to 
three  millimeters  in  thickness.  On  the  right  side  and 


60  FORMS  OF  NEUROSYPHILIS 

somewhat  anteriorly  opposite  the  junction  of  the  atlas 
and  axis  there  is  a  single  nodular,  firm  mass  which 
on  section  shows  a  yellowish,  firm  center  surrounded  by 
very  dense,  pearl-gray  tissue,  ^he  demarcation  be- 
tween the  homogeneous  yellowish  centre  and  its  sur- 
rounding gray  tissue  is  very  sharp.  This  nodule 
measures  about  0.75  to  I  cm.  in  diameter.  The  adjacent 
cord  is  deeply  indented  by  it.  Below  this  nodule  there 
is  a  translucent,  grayish  appearance  of  both  posterior 
sensory  columns  which  extends  downwards  and  di- 
minishes in  intensity  until  it  finally  disappears  ^  in  the 
upper  dorsal  region.  This  same  appearance  is  well 
marked  on  the  right  outer  margin  of  the  upper  cervical 
cord  corresponding  to  the  crossed  pyramidal  tract,  and 
extends  downwards  diminishing  in  intensity  until  it 
disappears  about  the  mid-dorsal  region.  The  left 
pyramidal  tract  appears  to  be  similarly  but  very 
slightly  involved;  section  of  the  lower  dorsal  cord  en- 
tirely negative.  Microscopically,  characteristic  GUMMA. 


It  is  a  question  whether  this  case  is  one  of  the  group  de- 
scribed in  1871  by  Charcot  under  the  name  of  pachymenin- 
gitis  cervicalis  hypertrophica.  Charcot  did  not  regard  his 
new  disease  as  syphilitic,  and  it  is  very  probable  that  syphilis 
is  not  responsible  for  all  cases.  Charcot,  however,  noted  that 
his  new  disease  was  not  incurable:  he  noted  that  the  re- 
sulting paraplegia,  although  it  might  be  very  marked  and 
accompanied  by  flexion  of  the  leg  on  the  thigh  and  although 
the  paraplegia  might  have  lasted  a  very  long  time,  might 
end  in  recovery.  Charcot  thought  that  surgical  intervention 
was  necessary.  He  described  three  periods  in  the  disease, 
the  first  or  neuralgic  (pseudo-neuralgic)  was  characterized  by 
sharp  pains  in  the  neck  and  by  the  sensation  of  constriction 
in  the  upper  part  of  the  thorax.  The  second  phase  of  the 
disease  was,  according  to  Charcot,  the  paralytic  phase,  in 
which  a  cervical  paraplegia  accompanied  by  muscular  atrophy 
developed.  Sometimes  cases  were  found  to  remain  in  this 
paralytic  phase  and  even  to  end  spontaneously  in  cure.  If 
the  muscular  atrophy  was  degenerative,  then  the  atrophy 
was  never  replaced;  but,  according  to  Charcot,  some  cases  of 
atrophy  were  simple  and  accordingly  curable.  If,  however, 


FORMS   OF   NEUROSYPHILIS  6l 

the  spinal  cord  itself  became  involved  in  the  meningeal 
inflammation,  then  phenomena  of  transverse  myelitis  set  in 
with  a  spastic  paraplegia  and  involvement  of  the  bladder  and 
rectum.  Muscular  atrophy  never  developed  in  the  legs,  at 
least  in  typical  cases. 

Among  the  causes  of  this  condition  the  following  have 
been  mentioned:  cold,  overexertion,  alcoholism,  tubercu- 
losis and  syphilis.  Syphilis  undoubtedly  plays  the  major 
part.  Even  before  the  days  of  the  W.  R.,  observers,  among 
whom  may  be  mentioned  Dejerine-Tinel  and  Pforringer, 
discovered  syphilis  in  nearly  all  sufferers  from  pachymenin- 
gitis  cervicalis  hypertrophica. 

It  should  be  differentiated  from  caries  of  the  spine  and 
cord  and  meningeal  tumors.  The  spinal  fluid  examination 
makes  this  somewhat  easy. 

Antisyphilitic  remedies  are  indicated,  and  should  be  tried 
even  when  the  etiology  is  obscure,  if  only  as  a  therapeutic 
test. 


But  what  have  been  thy  answers?    What  but  dark, 
Ambiguous,  and  with  double  sense  deluding, 
Which  they  who  asked  have  seldom  understood, 
And,  not  well  understood,  as  well  not  known? 

Paradise  Regained,  Book  I,  lines  434-437- 


II.  THE  SYSTEMATIC   DIAGNOSIS  OF  THE 
MAIN   FORMS   OF   NEUROSYPHILIS 


PARETIC  NEUROSYPHILIS  ("  general  paresis  ") 
sometimes  persistently  receives  the  diagnosis 
NEURASTHENIA  simply  through  omission  to 
apply  approved  diagnostic  methods. 


Case  9.  Greeley  Harrison,  a  man  of  46,  certainly  looked 
like  a  neurasthenic.  He  wanted  aid  for  nervous  indigestion 
of  years'  standing,  headache,  insomnia,  nervousness,  failing 
memory,  and  deafness.  He  volunteered,  in  fact,  that  he  had 
neurasthenia,  and  that  he  had  been  treated  for  this  by  hypo- 
phosphites. 

During  the  practically  negative  physical  examination,  Har- 
rison complained  of  headache  and  throbbing  in  the  head, 
and  during  examination  of  the  abdomen  felt  much  nauseated 
and  proceeded  to  vomit  rather  persistently.  There  were 
hemorrhoids. 

Neurological  examination  showed  that  the  left  pupil  was 
smaller  than  the  right,  was  irregular,  failed  to  react  con- 
sensually,  and  reacted  very  slowly  to  direct  light.  For  the 
rest,  however,  the  neurological  examination  was  negative. 
On  account  of  the  nausea  and  vomiting,  special  examination 
of  the  gastric  contents  was  made,  but  nothing  abnormal 
was  found. 

Mentally,  it  was  rather  striking  that  the  patient's  memory 
was  quite  inaccurate  both  for  remote  and  for  recent  events. 
His  school  knowledge  was  very  meagre.  As  for  delusions, 
the  only  approximation  thereto  was  the  patient's  continually 
dwelling  upon  his  bodily  symptoms.  Emotionally,  he  varied 
between  depression  and  a  sanguine  attitude. 

63 


64  SYSTEMATIC  DIAGNOSIS 

Although  there  was  no  symptom  directly  suggesting  syphi- 
lis in  the  Harrison  case,  the  slightly  abnormal  pupillary 
reactions  and  the  amnesia  warranted  the  suspicion  of  syphilis. 
The  blood  and  spinal  fluid  both  proved  positive  to  the  W.  R. ; 
the  gold  sol  reaction  was  of  the  "  paretic  "  type;  there  were 
18  cells  per  cmm.;  there  was  considerable  globulin,  and  an 
excess  of  albumin.  On  the  whole,  therefore,  we  felt  entitled 
to  make  the  diagnosis  GENERAL  PARESIS.  Why  should 
not  a  careful  observer  have  considered  syphilis  seriously? 
Yet  in  our  experience  such  cases  are  frequently  diagnosticated 
neurasthenia,  thus  entailing  dangerous  delay  in  treatment 
(in  this  case,  five  years'  delay). 

Going  over  the  history  of  the  case  with  still  greater  detail, 
we  learned  that  for  a  number  of  years  past,  there  had  been 
symptoms  of  a  neurological  nature.  For  instance,  five  years 
before,  at  the  age  of  41,  the  patient  had  been  apparently  over- 
come when  working  near  a  stove,  and  went  upstairs  talking 
incoherently,  but  recovered  shortly.  Thereafter,  such  spells 
occurred  almost  every  month;  later,  more  frequently;  still 
later,  the  attacks  were  associated  with  unconsciousness  and 
amnesia.  Occasionally  preceding  the  attack  there  would  be 
twitching  of  the  mouth,  jerking  of  the  arms,  and  incoherent 
talk.  Throughout  these  last  five  years,  in  point  of  fact,  the 
patient  had  been  unable  to  do  regular  work,  had  been  given 
to  much  complaining,  and  had  been  far  less  efficient  than 
formerly.  In  short,  it  would  seem  that,  with  the  improved 
technique  now  in  the  possession  of  medical  science  for  the 
diagnosis  of  general  paresis,  cases  like  that  of  Harrison  will  be 
diagnosticated  earlier  and  earlier. 

I.  How  typical  is  the  insidious  onset  of  symptoms  in  the 
case  of  Harrison?  The  onset  of  symptoms  in  neuro- 
syphilis  is  ordinarily  considered  to  be  sudden,  and  this 
statement  is  generally  true  despite  the  fact  that  after 
the  diagnosis  is  established  a  number  of  mild  prodro- 
mal symptoms  can  be  remembered  by  the  relatives. 
However,  some  cases,  of  which  Harrison  is  an  example, 
have  an  exceedingly  insidious  onset  without  sudden  ac- 
cess of  striking  symptoms.  Joffroy  and  Mignot  re- 
mark that  with  the  improvement  of  clinical  methods, 
the  course  of  paretic  neurosyphilis  must  now  be  stated 


SYSTEMATIC  DIAGNOSIS  65 

to  take  some  six  or  seven  years  for  completion.  In 
point  of  fact,  there  were  early  episodic  symptoms 
(seizures  almost  monthly)  which  should  not  have  es- 
caped medical  attention.  They  did  escape  medical 
attention,  however,  and  Harrison  was  wont  to  say 
"  Why  wasn't  I  told  that  my  disease  was  syphilis  five 
years  ago?" 

2.  Is  there  such  a  disease  as  syphilitic  neurasthenia?     Ac- 

cording to  Kraepelin,  syphilitic  neurasthenia  has  been 
described  as  occurring  shortly  after  infection  and  in 
the  first  stages  of  syphilis.  There  are  milder  and 
severer  forms;  the  milder  forms  show  discomfort,  diffi- 
culty in  thinking,  irritability,  insomnia,  cephalic  pres- 
sure, indefinite  variable,  uncomfortable  sensations,  and 
pains.  The  severer  cases  acquire  anxiety,  more  pro- 
nounced emotional  disorder,  dizziness,  disorder  of 
consciousness,  difficulty  in  finding  the  right  word, 
transient  palsies,  pronounced  sensory  disorders,  nausea, 
and  increase  of  temperature.  Kraepelin  is  in  doubt 
whether  there  is  any  definite  clinical  picture  of  this  sort, 
and  whether  there  is  any  causal  relation  between  the 
syphilitic  infection  and  such  symptoms  as  those  de- 
scribed. If  the  effect  of  knowledge  concerning  infection 
is  a  merely  psychic  effect,  then  it  is  improper  to  term 
the  neurasthenia  in  question  a  syphilitic  neurasthenia. 
For  the  relation  of  hysteria  to  the  acquisition  of  syphilis, 
see  below  the  case  of  Alice  Caperson  (46).  In  point  of 
fact,  modern  work  has  shown  even  in  the  primary  and 
secondary  stages  of  general  syphilis  more  or  less  pro- 
nounced neurosyphilitic  phenomena  in  the  shape  of 
the  so-called  meningitic  irritation  of  French  authors. 
(Besides  the  case  of  Caperson  (46),  see  the  case  of 
Fitzgerald  and  the  discussions  under  these  cases.) 

3.  What  is  the  relation  of  the  early  symptoms  of  this  case 

to  the  so-called  preparesis  of  Dana?  The  case  might 
well  have  been  an  example  of  Dana's  preparesis.  For 
a  discussion  of  this,  see  Case  of  William  Twist  (13). 

4.  What    is    the    classical    differential    diagnosis    between 

paretic  neurosyphilis  and  neurasthenia?  The  testing 
of  the  blood  by  the  W.  R.  is  unconditionally  necessary. 
If  the  W.  R.  is  negative,  the  diagnosis  of  paretic  neuro- 
syphilis is  extremely  improbable.  (It  must  be  borne  in 
mind  that  a  number  of  cases  of  paretic  neurosyphilis 
have  been  shown  to  have  a  negative  W.  R.  in  the  serum, 
and  receive  a  proper  diagnosis  only  after  spinal  fluid 
examination.)  Next  to  the  serum  W.  R.  stand  the 


66  SYSTEMATIC  DIAGNOSIS 

pupillary  and  aphasic  symptoms.  In  the  presence  of 
Argyll-Robertson  pupil  or  even  a  slight  speech  defect, 
the  diagnosis  of  neurasthenia  must  certainly  be  made 
with  caution  if  at  all.  Kraepelin  remarks:  The  sudden 
occurrence  of  neurasthenic  disorders  in  a  male  of 
middle  age  without  any  evident  cause  therefor  is  al- 
ways suspicious.  Yet  it  must  be  emphasized  that  a 
complaint  of  occasional  dizziness,  slight  speech  defect, 
tremor  of  tongue,  and  a  moderate  increase  of  tendon 
reflexes  do  not  possess  any  marked  diagnostic  sig- 
nificance. Clear  insight  and  understanding  of  the 
nature  of  the  disease  phenomena,  a  persistent  search 
for  recovery,  reasonableness  in  conversation,  progressive 
improvement  under  appropriate  treatment,  speak  for 
neurasthenia. 

Joffroy  and  Mignot  differentiate  what  they  call 
preparetic  neurasthenia  from  other  neurasthenic  states, 
not  only  on  the  basis  of  its  etiology  but  on  the  basis  of 
its  symptoms.  They  also  call  attention  to  the  fact  that 
neurasthenia,  being  a  pure  neurosis,  develops  either  on 
a  manifestly  hereditary  basis  or  upon  some  physical 
injury,  weakening  disease,  or  moral  shock.  The  pure 
neurotic  suffers  a  great  deal  more  than  the  patient  who 
is  destined  to  become  a  victim  of  paresis.  The  char- 
acter change  in  neurasthenia  does  not  amount  to  that 
entire  transformation  of  personality  (even  to  the  per- 
formance of  criminal  acts)  that  we  find  in  paretic 
neurosyphilis ;  at  the  most,  the  neurasthenic  shows 
minor  emotional  disturbances  and  a  certain  patho- 
logical egoism.  The  psychotherapeutic  test  also  rather 
readily  dissipates  many  of  the  neurotic,  hypochondriacal 
fears  and  feelings.  Although  both  pure  neurasthenia 
and  the  paretic  pseudoneurasthenia  are  characterized 
by  sexual  ^  weakness,  the  sexual  anaesthesia  of  the 
preparetic  is  practically  always  preceded  by  a  stage 
of  sexual  over-excitement.  These  finer  clinical  indi- 
cations, however,  fade  into  insignificance  beside  the 
data  that  can  and  should  be  obtained  from  laboratory 
tests. 

5.  How  exceptional  is  such  a  case  as  that  of  Harrison?  We 
have  in  our  experience  seen  many  patients  with  a 
similar  course  and  configuration  of  symptoms,  although 
the  majority  of  these  cases  in  a  community  advanced 
enough  to  provide  easy  access  to  a  Wassermann  labo- 
ratory are  now  diagnosticated  far  earlier  than  was  the 
case  of  Harrison. 


SYSTEMATIC  DIAGNOSIS  67 

6.  What  attitude  shall  we  take  toward  so-called  syphilo- 
phobia?  It  seems  to  us  that  resort  to  a  serum  W.  R. 
is  indicated,  both  from  the  standpoint  of  the  community 
and  still  more  importantly  from  the  standpoint  of  the 
patient.  We  are  even  inclined  to  suggest  for  a  case  of 
persistent  syphilophobia,  when  the  serum  W.  R.  has 
proved  negative,  a  lumbar  puncture.  Syphilophobia 
must  be  considered,  not  as  a  syphilitic  psychosis,  but 
as  a  phobia  to  be  classified  among  the  psychoneuroses. 
It  becomes  a  difficult  question  to  decide  at  times 
whether  a  patient  who  has  had  syphilis,  has  had  a 
considerable  course  of  treatment  and  shows  the  symp- 
toms of  a  syphilophobiac  should  be  further  treated  for 
syphilis  or  merely  for  his  phobia.  We  have  seen  re- 
cently such  a  patient  who  gave  a  certain  history  of 
syphilis  and  who  was  greatly  disturbed  lest  he  should 
be  developing  paresis.  This  fear  bothered  him  greatly. 
Examination  showed  irregular  pupils,  but  no  other 
signs  of  syphilis.  The  W.  R.  in  blood  and  spinal  fluid 
was  negative  as  were  the  other  spinal  fluid  tests.  It 
was  considered  wise  to  treat  him  only  for  his  phobia 
and  under  this  treatment  he  was  given  some  relief. 


58  SYSTEMATIC  DIAGNOSIS 


PARETIC  NEUROSYPHILIS  ("  general  paresis  ") 
may  look  precisely  like  MANIC-DEPRESSIVE 
PSYCHOSIS. 


Case  10.  The  mental  picture  in  Lyman  Agnew,  an  archi- 
tect, 58  years  of  age,  was  wholly  characteristic  of  manic- 
depressive  psychosis.  In  the  first  place,  there  had  been  (at 
55)  a  previous  attack  of  depression,  lasting  a  few  months, 
from  which  Agnew  had  completely  recovered.  He  had 
remained  entirely  well  up  to  four  months  before  consultation. 
(Manic-depressive  psychosis  is,  at  least  in  a  majority  of 
cases,  hereditary.  There  had  been  mental  disorder  in  one 
maternal  cousin,  and  mental  impairment  in  the  patient's 
mother  some  time  before  her  death  from  cerebral  hemorrhage. 
There  was  no  other  report  of  mental  disease  in  the  family.) 

It  appears  that  in  the  interval  between  attacks,  Agnew 
had  been  working  very  hard  and  had  been  fairly  successful 
in  paying  off  a  mortgage  on  his  house.  A  marked  elation, 
somewhat  natural,  followed  this  success  and  continued  to  an 
abnormal  degree.  Agnew  labored  under  considerable  ex- 
citement, was  over-fussy,  and  at  times  showed  a  flight  of 
ideas.  His  mania  or  hypomania  gradually  diminished  and 
depression  set  in,  in  which  depression  he  arrived  for  consulta- 
tion. He  had  marked  ideas  of  self-accusation,  was  emotion- 
ally unstable,  wept  much,  and  showed  a  characteristic 
retardation  of  activities  and  unrest. 

Physically,  there  was  no  neurological  disorder.  The  patient 
appeared  rather  under-nourished.  The  heart  borders  lay 
2  cm.  to  the  right  and  at  nf  cm.  to  the  left  of  the  mid- 
sternal  line.  The  aortic  second  sound  was  very  loud.  There 
was  a  moderate  radial  arteriosclerosis.  Systolic  blood  pres- 
sure was  210,  diastolic  155. 

The  high  blood  pressure  suggested  nephritis,  possibly  of 
arteriosclerotic  origin,  but  urine  examination  and  blood- 
nitrogen  tests  yielded  no  evidence  of  kidney  disease.  More- 
over, it  is  our  experience  that  a  manic-depressive  psychosis 


SYSTEMATIC  DIAGNOSIS  69 

in  persons  past  middle  life  is  not  infrequently  complicated 
by  high  blood  pressure.  In  point  of  fact,  some  authors  in- 
sist upon  a  relation  between  manic-depressive  psychosis  and 
the  arteriosclerosis  which  rather  frequently  sets  in  in  this 
disease. 

Routine  examination  of  the  blood  serum,  however,  yielded 
a  positive  W.  R.  Following  the  approved  rule  of  making  an 
examination  of  the  spinal  fluid  in  all  mental  cases  having  a 
positive  serum  W.  R.,  we  proceeded  to  lumbar  puncture.  The 
fluid  was  clear  and  contained  35  cells  per  cmm.,  the  albumin 
was  in  excess,  and  there  was  a  positive  globulin  reaction. 
The  gold  sol  reaction  was  of  the  "  paretic  "  type;  the  W.  R. 
was  strongly  positive. 

On  this  basis,  it  seems  worth  while  to  consider  the  diag- 
nosis of  GENERAL  PARESIS  or  that  of  some  form  of  non- 
paretic  neurosyphilis.  The  former  is  the  diagnosis  which  we 
prefer. 

1.  What  is  the  classical  differential  diagnosis  between  manic- 

depressive  psychosis  and  neurosyphilis?  The  labora- 
tory tests  have  naturally  supplanted  the  older  purely 
clinical  methods  of  differential  diagnosis.  The  diffi- 
culties lodge,  in  the  first  instance,  in  depressive  states. 
It  would  appear  to  be  impossible  on  purely  clinical 
grounds  in  certain  cases  to  tell  the  depression  of  neuro- 
syphilis from  the  depression  of  manic-depressive  psy- 
chosis, since  the  slightly  greater  interest  in  the  outer 
world  taken  by  manic-depressive  patients  and  their 
greater  responsiveness  to  diagnostic  threats  (suggestion 
that  patient  is  to  be  pinched  or  cut)  are  of  no  special 
value  in  the  individual  case.  Identical  considerations 
hold  for  the  maniacal  phases  of  manic-depressive  psy- 
chosis, for  these  maniacal  phases  may  even  develop 
delusions  (Kraepelin)  of  precisely  the  same  nature  as  the 
characteristic  expansive  delusions  of  the  excited  paretic. 

2.  If  the  clinical  symptoms  are  insufficient  in  differential 

diagnosis,  are  not  the  pupillary  signs  and  the  speech 
defect  of  greater  value?  They  are  of  value  if  present, 
but  as  in  the  case  of  Agnew,  the  victim  of  neurosyphilis 
may  show  no  pupillary  or  speech  disorder.  Instances 
are  familiar,  also,  in  which  the  pupillary  and  speech 
signs  are  absent  in  very  advanced  cases  of  non-paretic 
or  even  of  paretic  neurosyphilis. 


SYSTEMATIC  DIAGNOSIS 

Would  not  a  circular  course  or  recurrence  of  attacks 
be  decisive  for  manic-depressive  psychosis?  Paretic 
neurosyphilis  sometimes  exhibits  the  same  circular 
or  recurrent  course.  We  conclude  that  neither  the 
clinical  symptoms,  the  classical  pupillary  and  speech 
signs,  nor  the  ups  and  downs  of  a  particular  disease,  are 
at  all  decisive  as  between  manic-depressive  psychosis 
and  paretic  neurosyphilis.  Resort  must  be  had  to 
laboratory  tests. 

What  is  the  significance  of  the  high  blood  pressure  in 
paretic  neurosyphilis?  Work  from  our  laboratory 
(Southard  and  Canavan)  has  shown  plasma  cells  in 
the  kidneys  in  17  out  of  30  paretics  (56%),  and  in 
1 6  of  these  17  paretics  with  renal  plasmocytosis,  the 
plasma  cells  were  found  in  the  periglomerular  region. 
What  the  relation  of  these  findings  may  be  to  heightened 
blood  pressure  is  as  yet  unknown.  The  severe  syphi- 
litic involvement  of  the  aorta  so  characteristic  in  paretic 
neurosyphilis,  as  in  other  forms,  may  possibly  have  a 
bearing  on  blood  pressure. 


SYSTEMATIC  DIAGNOSIS  71 


A  POSITIVE  SERUM  WASSERMANN  REAC- 
TION associated  with  mental  symptoms  (even 
with  grandiosity)  does  NOT  prove  the  EXIST- 
ENCE OF  PARETIC  NEUROSYPHILIS  ("  gen- 
eral paresis  "). 


Case  ii.  Juliette  Lachine  came  to  a  general  hospital 
with  pain  in  the  right  upper  quadrant  of  the  abdomen,  wherein 
was  found  an  enlarged  liver.  This  liver  was  regarded  as 
syphilitic  on  the  ground  that  the  patient  had  a  positive  serum 
W.  R.  and  that  her  two  elder  children  were  clearly  suffering 
from  congenital  syphilis.  The  liver  mass  was  promptly  re- 
duced by  antisyphilitic  treatment  of  the  classical  sort. 
When,  however,  the  patient  was  given  an  injection  of  salvar- 
san,  she  shortly  began  to  develop  marked  mental  symptoms, 
whereupon  she  was  removed  to  the  Psychopathic  Hospital. 

The  mental  picture  at  the  Psychopathic  Hospital  was  as 
follows:  Lack  of  orientation  for  time,  marked  distractibility 
of  attention,  with  a  certain  jumping  from  one  subject  to 
another,  delusions  of  a  religious  nature,  claims  of  wonderful 
powers  possessed  by  the  patient,  moods  variable,  though  as  a 
rule  of  a  euphoric  and  elated  nature,  with  laughing  and  sing- 
ing. The  activity  seemed  to  be  of  a  mental  rather  than  a 
peripheral  nature.  The  patient  did  not  regard  herself  as 
mentally  abnormal.  The  liver  was  still  4  cm.  below  the  costal 
margin  in  the  nipple  line.  We  found  the  W.  R.  to  be  positive 
in  the  serum  but  negative  in  the  spinal  fluid.  In  fact,  the 
spinal  fluid  was  entirely  negative. 

So  far  as  we  are  aware  the  picture  presented  by  this  case 
is  one  of  MANIC-DEPRESSIVE  PSYCHOSIS.  We  regard  the  dis- 
ease as  merely  complicating  the  syphilis,  although  it  is  en- 
tirely possible  that  some  visceral  condition  incidental  to  the 
syphilis  might  be  proved  (in  a  higher  stage  of  psychiatric 
science)  to  have  produced  the  mania. 

In  any  event,  the  patient  quite  recovered  from  her  mental 
symptoms  in  a  month.  She  was  then  able  to  tell  us  of  a 


72  SYSTEMATIC  DIAGNOSIS 

previous  attack  of  depression  some  12  years  previously, 
namely,  at  the  age  of  26.  It  apears  that  she  had  at  that  time 
been  committed  to  a  hospital  for  the  insane. 

1.  In  this  case,  in  which  the  diagnosis  of  manic-depressive 

pyschosis  and  not  paretic  neurosyphilis  was  made, 
are  we  sure  that  the  symptoms  that  we  term  manic-de- 
pressive psychosis  were  not  actually  produced  by 
syphilotoxins?  In  other  words,  in  the  absence  of 
spinal  fluid  signs  of  inflammation  or  chemical  change, 
might  it  not  be  possible  for  generalized  syphilis  out- 
side the  nervous  system  to  produce  manic-depressive 
symptoms?  There  is  so  far  in  the  literature  no  experi- 
mental or  other  evidence  of  syphilotoxins.  The  ex- 
istence of  products  and  substances  permitting  the  W.  R. 
and  the  gold  sol  reaction  is  not  of  course  evidence  of 
syphilotoxins.  Although  there  is  no  evidence  of  soluble 
syphilotoxins,  it  is  thought  that  in  the  so-called  Jarisch- 
Herxheimer  reaction  (the  intensification  of  clinical 
symptoms  after  salvarsan  injection)  effects  may  be 
due  to  the  liberation  of  products  from  the  killed  bodies 
of  spirochetes.  Such  endotoxins  are  not  here  in 
question. 

2.  Is  visceral  syphilis,  such  as  gumma  of  the  liver,  able  to 

produce  characteristic  syphilitic  reactions  in  the  spinal 
fluid?  We  have  had  an  autopsied  case  in  which  there 
was  a  "  paretic  "  gold  sol  reaction  of  the  fluid  (though 
without  other  signs).  The  autopsy  showed  gummata 
of  the  liver.  However,  the  finer  anatomy  of  the  ner- 
vous system  showed  a  mild  but  definite  meningo-en- 
cephalitic  process,  which  was  doubtless  responsible 
for  the  gold  sol  reaction. 

3.  What  is  the  value  of  grandiose  ideas?     Ballet  distin- 

guishes two  groups  of  grandiose  ideas :  (a)  ideas  of  self- 
satisfaction,  including  ideas  concerning  extraordinary 
capacity,  strength,  power,  and  wealth  on  the  part 
of  the  patient;  and  (5)  ideas  of  ambition;  the  latter 
being  of  a  more  exact,  constant,  uniform  and  systema- 
tizing nature.  The  more  vague  and  less  systematized 
ideas  of  self-satisfaction  rest  in  a  phase  of  contentedness 
and  optimism;  the  more  definite  ideas  of  pride  and 
ambition  are  responsible  for  striking  transformations 
of  personality.  General  paresis  shows,  according  to 
Ballet,  these  ideas  of  self-satisfaction  in  their  most 
developed  form.  A  certain  variability,  absurdity,  in- 


SYSTEMATIC  DIAGNOSIS  73 

coherence,  and  contradictoriness  characterize  these 
ideas  and  the  patient  has  little  or  no  insight  into  their 
nature.  When  such  ideas  occur  at  the  outset  of  the 
disease,  they  naturally  may  be  of  medico-legal  interest. 
Cotard  explains  these  ideas  of  megalomania  on  the  part 
of  paretics  on  the  ground  that  they  are  essentially  motor 
or  will  disorders  and  rest  upon  a  sort  of  hyperbulia, 
exhibiting  itself  in  exuberant  activity.  Regis  has 
thought  that  the  delusional  generosity  and  liberality 
of  the  paretic,  and  his  willingness  to  lend  his  wealth 
and  talents  to  social  progress,  is  helpful  for  diagnosis 
when  contrasted  with  the  more  personal  egoism  of  the 
victim  of  manic-depressive  psychosis.  The  self-satis- 
faction of  the  manic-depressive  patient  often  does  not 
reach  a  delusional  stage,  but  remains  a  mere  feeling  of 
pathological  well-being  or  euphoria.  The  maniacal 
patient  may  compare  himself  with  some  great  man  but 
he  does  not  identify  himself  with  him.  It  must  be 
remembered  that  these  ideas  of  self-satisfaction  occur 
also  in  alcoholism,  but  according  to  Ballet  they  occur 
only  in  the  dementing  phase  of  chronic  alcoholism, 
and  have  no  special  diagnostic  value.  They  may  be 
a  clinical  stumbling-block  for  a  time  in  the  cases  of 
alcoholic  pseudoparesis.  As  for  the  ideas  of  am- 
bition in  which  the  patients  believe  themselves  to  be 
princes,  emperors,  divine  messengers,  and  the  like,  these 
are  less  characteristic  of  paretic  neurosyphilis  than  of 
delusional  psychoses  of  a  non-syphilitic  nature.  At 
all  events,  such  ideas  if  definite,  of  long  standing, 
and  systematized  by  the  patient  to  form  a  thorough- 
going portion  of  his  life,  are  not  characteristic  of  neuro- 
syphilis. The  victim  of  paretic  neurosyphilis  can  as  a 
rule  be  persuaded  out  of  his  delusions,  at  least  for 
the  time  being.  These  distinctions,  it  must  be  added, 
are  hardly  of  value  in  the  early  cases  of  any  of  the 
psychoses  in  question,  and  cannot  be  made  as  a  rule  in 
either  private  or  psychopathic  hospital  practice.  Typi- 
cal examples  of  grandiosity,  although  not  so  frequent 
as  might  be  thought  from  textbooks,  are  always  on  dis- 
play in  institutions  for  the  chronic  insane. 


74  SYSTEMATIC  DIAGNOSIS 


PARETIC  NEUROSYPHILIS  ("general  pare- 
sis") may  look  precisely  like  DEMENTIA 
PRAECOX.  Autopsy. 


Case  12.  Henry  Phillips  remains  a  striking  case  in  the 
memory  of  those  who  knew  him  and  his  medical  findings. 
Phillips  came  to  the  hospital  voluntarily  at  42  years  of  age 
from  the  bank  where  he  worked  as  a  clerk;  he  came  at  the 
suggestion  of  his  employer.  It  seems  that  he  had  been 
annoying  his  associates  because  he  had  fallen  into  a  habit  of 
continually  scratching  himself.  Phillips  was  entirely  sure 
that  he  was  the  victim  of  what  he  called  the  "  Scotch  itch," 
and  explained  off-hand  that  this  itch  had  been  put  upon  him 
by  the  Free  Masons  as  a  matter  of  revenge  because  he  would 
not  join  their  order.  He  said  once,  for  example:  "  At 
times  I  feel  like  raising  Hell ;  then  I  get  a  psychic  intimation ; 
and  then  I  get  to  using  a  foot-rule  on  my  back  and  to  slapping 
my  face."  He  explained  this  psychic  intimation  as  coming 
from  the  order  of  Scottish  Rites.  Another  example  of  talk  is 
as  follows:  "  My  father  is  a  fighting  man;  that  is  part  of  it. 
They  mean  to  throw  me  down.  I  am  through  now  trying 
for  membership  in  the  Free  Masons.  They  have  good  cause, 
they  must  fight.  They  do  not  want  me  for  some  personal 
matters.  I  can  go  just  so  far  in  agreeing  and  seconding  their 
advances,  but  in  the  end  it  fails.  I  have  no  strength  nor 
endurance." 

Aside  from  these  delusions,  there  was  little  abnormality 
to  be  found,  though  his  recollection  for  minor  events  of  the 
immediate  present  was  inaccurate.  He  was  rather  abnor- 
mally impulsive,  gesticulating  a  good  deal  while  talking, 
and  was  of  the  appearance  that  the  laity  call  "  nervous." 
It  appears  that  he  had  always  been  peculiar,  subject  to 
violent  fits  of  temper,  in  which  fits  he  might  throw  things  at 
other  members  of  the  family.  He  always  had  pronounced 
likes  and  dislikes  which  he  never  concealed.  He  had  never 
had  friends,  had  always  been  secretive;  and  he  was  often 


SYSTEMATIC  DIAGNOSIS  75 

termed  a  great  student.  For  some  five  years  he  had  been 
studying  Japanese  from  time  to  time,  associating  himself 
with  a  Japanese. 

It  never  does  to  jump  at  the  diagnosis  dementia  praecox. 
However,  the  picture  seemed  characteristic  enough  for  the 
paranoid  form  of  this  disease.  Physically,  Phillips  had 
no  particular  abnormality;  the  knee-jerks  were  a  little  lively, 
and  the  pupils  reacted  a  little  sluggishly.  However,  the 
routine  W.  R.  of  the  serum  proved  to  be  positive.  Exami- 
nation of  the  spinal  fluid  was  resorted  to,  —  as  in  all  cases 
with  a  positive  serum  W.  R.  —  and  it  also  proved  to  be 
positive  and  strongly  so;  the  globulin  and  albumin  were  in- 
creased, and  there  was  a  pleocytosis.  A  diagnosis  of  neuro- 
syphilis  was  hardly  avoidable.  Phillips  later  admitted  a 
chancre,  which  he  claimed  was  located  on  the  mucous  mem- 
brane of  the  cheek  and  acquired  by  using  the  same  utensils 
as  his  Japanese  friend,  which  friend,  he  stated,  had  active 
syphilis. 

Antisyphilitic  treatment  of  considerable  intensiveness 
was  begun,  with  intravenous  injections  of  salvarsan  and 
intraspinous  injections  of  salvarsanized  serum,  but  the 
patient  grew  steadily  worse.  His  mental  symptoms  became 
more  marked,  although  not  especially  characteristic  of  general 
paresis.  Neurologically,  he  did  develop  signs  more  sugges- 
tive of  general  paresis,  and  18  months  later  died. 

The  autopsy  showed  features  of  GENERAL  PARESIS.  It  is 
not  necessary  to  enter  into  the  question  of  the  details  of  his- 
tological  correlation  at  this  time. 

1.  What  conclusion  can  be  drawn  from  lively  knee-jerks? 

Lively  knee-jerks  are  of  very  little  significance.  Not 
only  certain  newrosyphilitics  but  also  a  variety  of  neu- 
rotic persons,  victims  of  dementia  praecox  and  hysteria, 
are  very  prone  to  have  active  tendon  reflexes.  Of 
course,  extreme  degrees  of  exaggeration  are  of  import- 
ance, and  especially  an  association  of  the  hyperreflexia 
with  the  Babinski  reaction,  the  Gordon,  or  Oppenheim 
reflexes,  ankle  clonus,  and  the  like. 

2.  Is  there  any  special  or  differentiating  factor  in  an  extra- 

genital  chancre  as  against  a  genital  chancre?     Prob- 


76  SYSTEMATIC  DIAGNOSIS 

ably  this  question  should  be  answered  in  the  negative. 
Some  have  claimed  that  chancres  draining  by  lymphatic 
channels  of  the  head  are  more  likely  to  lead  to 
cerebral  syphilis.  This  idea  cannot  be  said  to  be 
established. 

3.  Is  there  any  significance  in  the  story,  if  true,  that 
Phillips  acquired  his  syphilis  from  a  Mongolian?  It 
seems  to  be  fairly  well  established  that  syphilis  of  the 
nervous  system  is  extremely  rare  in  China  and  Japan, 
whereas  bone  syphilis  is  very  frequent  there.  It  has 
been  held  that  this  has  to  do  (a)  with  strains  of 
spirochetes,  (&)  with  the  state  of  civilization,  or  (c) 
with  the  degree  of  "  syphilization."  Apparently  when 
a  race  is  first  infected  with  syphilis  the  lesions  are 
chiefly  of  the  cutaneous  and  osseous  systems;  only  in 
later  generations  the  vascular  and  nervous  systems 
suffer.  However,  involvement  of  the  nervous  systems 
of  Mongolians  resident  in  this  country  is  no  rarity,  a 
point  possibly  in  favor  of  the  theory  of  special  strains 
affecting  the  nervous  system  as  prevalent  in  western 
countries.  Little  or  nothing  is  known  as  to  the  effect 
of  transmission  from  one  race  to  another,  as  from 
Mongolian  to  Caucasian  in  Phillips'  story. 


SYSTEMATIC  DIAGNOSIS  77 


NEUROSYPHILIS  is  NOT  to  be  entirely  ruled  out 
by  a  negative  serum  Wassermann  Reaction;  for 
the  fluid  Wassermann  Reaction  may  be  positive. 


Case  13.  William  Twist  is  a  case  of  note  in  the  matter 
of  the  so-called  preparetic  period  (the  idea  of  Charles  L. 
Dana  which  was  scoffed  at  when  first  proposed  by  him  in  1910). 
The  patient,  a  very  successful  traveling  salesman,  35  years 
of  age,  was  admitted  to  the  Psychopathic  Hospital  showing 
a  typical  picture  of  general  paresis. 

Thus,  mentally,  the  patient  showed  elation,  grandiosity 
(millions  of  dollars  to  give  away),  intellectual  weakness, 
disorder  of  memory,  lack  of  judgment,  rambling  talk,  speech 
defect,  omission  of  letters  in  writing  and  spelling. 

Neurologically,  there  was  tremor  of  the  lips,  slighfctfrregu- 
larity  of  the  pupils,  which  however  reacted  well,  and  lively 
knee-jerks. 

Mr.  Twist  had  sought  advice  at  our  out-patient  department 
in  his  thirty-third  year.  The  records  show  that  at  that 
time  he  was  somewhat  depressed,  and  his  speech  was  even 
then,  according  to  his  own  statement,  stammering.  However, 
we  found  the  W.  R.  at  that  time  to  be  negative  in  the  blood 
serum.  It  appeared  that  his  mother  had  died  of  consump- 
tion; his  father  was  said  to  have  committed  suicide.  A 
brother  had  once  recovered  from  an  attack  of  depression, 
presumably  an  attack  of  manic-depressive  psychosis.  Ac- 
cordingly, we  thought  at  the  time  that  the  case  was  probably 
one  of  manic-depressive  psychosis.  Moreover,  our  routine 
serum  W.  R.  failed  to  indicate  any  syphilitic  process.  As  for 
the  so-called  stammering  of  speech,  this  appeared  to  be  a 
matter  of  the  patient's  own  recollection  rather  than  of  our 
observation.  In  any  event,  the  patient  had  gone  into  the 
country  and  appears  to  have  entirely  recovered ;  falling,  again, 
however,  into  mental  difficulties  after  a  short  period,  and 
finally  arriving  at  the  hospital  in  the  above-mentioned  classi- 
cal condition. 


78  SYSTEMATIC  DIAGNOSIS, 

^« 

The  W.  R.  in  the  blood  serum  proved  again  negative. 
The  test  was  repeated  a  number  of  times ;  also,  after  salvar- 
san  had  been  given.  The  salvarsan  did  not  act  provoca- 
tively, and  the  blood  serum  has  remained  consistently  nega- 
tive. 

In  cases  of  syphilis  the  W.  R.  is  at  times  negative.  Swift 
claims  that  in  such  cases  an  injection  of  salvarsan  will  often 
produce  a  positive  W.  R.  if  the  blood  is  tested  on  several  days 
following  the  injection. 

The  spinal  fluid,  however,  did  show  a  positive  W.  R.  as 
well  as  a  gold  sol  reaction  of  a  "  paretic  "  type.  There  were 
at  the  first  examination  194  cells  per  cmm.,  there  was  a  mod- 
erate excess  of  albumin,  and  a  positive  globulin  test.  In 
short,  there  was  no  question  of  any  other  diagnosis  than 
GENERAL  PARESIS. 

1.  How  can  the  negative  W.  R.  of  the  blood  serum  be  ex- 

plained? It  is  difficult  or  impossible  to  explain  this. 
Figures  differ  as  to  the  percentage  of  cases  of  general 
paresis  with  negative  blood  serum ;  perhaps  3  to  5%  of 
these  cases  yield  a  negative  serum  W.  R. 

It  is  important  to  note  the  long  preparetic  period :  at 
least  a  year  and  a  half.  Could  our  diagnostic  methods 
be  sharpened  a  trifle,  such  cases  as  these  could  be  ob- 
tained early  in  this  preparetic  period  and  it  might 
then  be  safe  to  promise  good  therapeutic  results. 

2.  What  is  the  nature  of  the  preparesis  of  Dana?     When 

Dana's  brief  paper  on  preparesis  was  written,  there 
was  of  course  hardly  any  idea  that  cases  of  paretic 
neurosyphilis  could  be  cured  or  would  recover,  except 
possibly  vanishingly  few  cUriosa  about  which  there 
would  always  rage  a  diagnostic  question.  Accordingly, 
Dana,  having  found  certain  cases  that  seemed  to  him 
to  have  early  signs  of  paresis  but  had  apparently  been 
cured  by  treatment,  proposed  to  call  them  cases  of 
preparesis.  His  idea  was  that  he  would  thereby  not 
offend  those  who  held  that  general  paresis  was  theo- 
retically a  fatal  disease.  With  modern  work  and  the 
display  of  more  and  more  atypical  cases  of  neuro- 
syphilis, and  the  observation  of  relatively  numerous 
cures  or  remissions  under  treatment,  the  designation 
of  preparesis  for  a  separate  entity,  or  even  for  a  sub- 
form  of  neurosyphilis,  becomes  superfluous. 


SYSTEMATIC   DIAGNOSIS  79 

3.  What  is  the  percentage  of  cases  of  paretic  neurosyphilis 

that  show  a  negative  serum  W.  R.?  Among  the  best 
figures  are  those  of  Miiller,  who  found  that  of  386  ex- 
amples of  paretic  neurosyphilis,  379  showed  all  reac- 
tions positive,  or  98.5%. 

4.  What  is  the  meaning   and  value  of  the  so-called   pro- 

vocative salvarsan  injection?  In  practice,  there  may 
be  a  series  of  negative  W.  R.'s  in  the  blood  serum 
before  a  positive  reaction  is  finally  obtained,  owing  to 
technical  difficulties  or  biological  peculiarities.  Where 
intensive  work  is  being  done  upon  the  neurosyphilis 
problem,  it  is  beyond  question  desirable  to  make  the 
W.  R.  test  upon  at  least  three  separate  samples  of  blood 
drawn  at  intervals,  for  the  second  or  third  test  may 
prove  positive.  This  situation  makes  the  interpretation 
of  the  so-called  provocative  salvarsan  injection  exceed- 
ingly doubtful ;  that  is,  the  reaction  might  have  been 
positive  on  repetition  without  the  injection  of  salvarsan. 
The  present  case,  as  above  stated,  failed  to  yield  a  serum 
W.  R.  even  after  repeated  tests  and  the  "  provocative." 
J5.  What  is  the  significance  of  the  irregular  pupils  in  this 
group?  Paretic  neurosyphilis  shows  inequality  of  the 
pupils  in  a  high  per  cent  of  cases.  Irregularity  of  out- 
line of  the  pupils  is  commonly  thought  to  be  an  im- 
portant sign  and  to  suggest  neurosyphilis.  It  is  true 
that  many  cases  of  pupillary  irregularity  are  syphilitic, 
but  the  sign  is  of  little  or  no  differential  value  since 
congenital  malformations  and  relics  of  old  injuries  and 
adhesions  may  produce  effects  identical  with  those  of 
neurosyphilis. 


g0  SYSTEMATIC   DIAGNOSIS 


DIFFUSE  (that  is,  meningovasculoparenchyma- 
tous*)  NEUROSYPHILIS  is  typically  associated 
with  six  positive  tests  (serum  Wassermann  reaction, 
fluid  Wassermann  reaction,  spinal  fluid  gold  sol 
reaction,  pleocytosis,  positive  globulin,  excessive 
albumin);  but  one  or  more,  and  frequently  sev- 
eral, of  these  tests  are  likely  to  run  mild  as 
compared  with  the  tests  in  PARETIC  NEURO- 
SYPHILIS ("general  paresis").  ,The  clinical  course 
of  the  diffuse  (and  especially  the  meningovascular) 
cases  is  likely  to  be  protracted,  with  a  good  prog- 
nosis as  to  life  (barring  fatal  vascular  insults). 


Case  14.  We  shall  present  the  case  of  John  Jackson,  a 
surveyor,  31  years  of  age,  suffering  from  a  left  hemiplegia, 
with  this  in  mind:  To  exhibit  difficulties  in  diagnosis  in  the 
presence  of  an  embarrassment  of  symptomatic  riches. 

The  patient  arrived  at  the  hospital,  in  the  first  place,  be- 
cause he  had  been  threatening  a  woman  who  lived  next  door 
to  him.  He  believed  that  this  neighbor  had  been  talking 
about  him  and  circulating  reports  against  him.  Excited  by 
these  ideas,  he  had  threatened  to  cut  her  throat. 

Now  the  occurrence  of  hemiplegia  in  adult  life  before  the 
approach  of  senium  is  always  suspicious  of  syphilis,  and  this 
suspicion  we  naturally  entertained  from  the  beginning. 
However,  there  was  upon  the  scalp  a  crooked  linear  furrow 
about  six  inches  long,  running  from  the  vertex  to  the  right 
parietal  eminence.  Another  furrow  about  an  inch  long 
was  present  upon  the  forehead.  These  furrows  appeared  to 
be  of  a  bony  nature  and  were  not  tender.  There  was  evi- 
dence of  an  old  decompression  operation  on  the  right  side  of 
the  head;  there  were  also  large  scars  on  both  sides  of  the 

*  Proof  of  marked  parenchymatous  lesions  must  hang  on 
post-mortem  data;  the  inference  here  as  to  the  presence  of 
parenchymatous  lesions  is  a  clinical  inference. 


Station  in  syphilitic  hemiplegia. 
Syphilitic  pigmentation  of  skin. 


SYSTEMATIC  DIAGNOSIS  8 1 

neck,  evidently  the  result  of  old  operations;  and  there  were 
numerous  palpable  glands  —  the  largest  about  the  size  of 
a  lima  bean  —  all  firm  and  not  tender. 

It  seems  that  at  the  age  of  eight,  according  to  the  patient's 
mother,  Jackson  had  received  a  head  injury  and  had  re- 
mained unconscious  for  three  weeks.  Upon  recovery,  he 
had  to  relearn  both  to  walk  and  to  talk;  however,  he  was 
able  to  begin  school  where  he  left  off.  He  became  more  ner- 
vous and  irritable  after  the  accident  than  previously.  Noth- 
ing further  had  developed  until,  at  about  25  years  of  age,  a 
tubercle  was  discovered  in  his  eye  (the  right  pupil  was 
smaller  than  the  left,  reacting  more  slowly;  right  iris  bound 
down  by  adhesions,  with  white  opacity  of  anterior  chamber). 
For  two  years,  25  to  27,  the  patient  was  under  medical 
treatment  for  tuberculosis,  and  at  the  conclusion  of  this 
period  numerous  glands  were  removed  from  the  neck  and 
diagnosticated  tuberculous.  However,  the  neck  did  not 
heal  and  he  carried  bandages  upon  it  for  two  years. 

At  28,  the  patient's  mother  described  the  occurrence  of 
a  slight  shock,  with  head  retraction,  for  a  minute  or  two, 
and  inability  to  speak.  Thereafter  there  had  been  five  or  six 
similar  attacks,  less  severe,  and  without  loss  of  speech.  The 
attacks  were  never  accompanied  by  convulsive  movements. 
Then  occurred  a  paralytic  stroke,  leaving  the  patient  with  a 
left  hemiplegia,  which  had  somewhat  improved.  Mentally, 
the  patient  had  gone  down  hill,  becoming  less  alert  and  more 
apathetic,  and  to  some  extent  amnestic.  One  had  to  con- 
sider, accordingly,  the  somewhat  doubtful  possibility  of 
post-traumatic  and  post-operative  conditions,  and  the  ques- 
tion of  tuberculosis  (possibly  errors  in  diagnosis;  the  lungs 
showed  no  evidence  of  tuberculosis). 

Physically,  the  signs  of  a  left  hemiplegia  were  appropriate. 
Spasticity  on  the  left  side  was  found;  there  were  Babinski, 
Gordon,  Oppenheim  reflexes  and  ankle  clonus  on  the  left 
side  (all  absent  on  the  right).  Speech  defect  was  present. 
Mentally,  aside  from  the  delusions  noted  at  the  beginning  of 
our  analysis,  a  striking  feature  was  the  patient's  childishness. 
While  reciting  delusions,  the  patient  was  overactive  and 
evinced  a  somewhat  childish  interest.  Arithmetically,  Jack- 


82 


SYSTEMATIC   DIAGNOSIS 


M 

W 

.ON                    -                                  £_,(/} 

w~ 

53 
o 

W    f_                                                 S     ^ 

oo 

I 

§ 

g. 

PLEOCYT 

Eg      E  65      £  3  § 

CD    5                 1/5     2.                 ^    rf"    S< 

o  <         o  °"         o  ^  <: 

OH                          PH                           P-.    ' 

H 

H 
«{ 

U 

1 

g           5 

i 

HH-     2 

J 

^^            ^t^»            ^  ,    ,   .«  H         PH^ 

1UROSYP 

W    u 

31 

OH  (j 

OH                         OH                         AH 

m 

K 

a 

5 

5 

W                  U                  W  [o  S 

>   0        >   0        >  o  < 

rf\ 

J     O 

E~*  ^^            E~"   "^            F~<   S   ^ 

O 

B 

1  " 

g    §             g    §             g   ^   < 

OH                          CU                          OH 

| 

u   S 

*"*     £3 

... 

{H 

(S     M 

N     W 

>    &?                 [>                          ^    ^? 

M 

O 

i 

d  w 

r  Q 
-J    o 

w  o 

£      0                  £<                          *-H      0 
H      ON                 f-H    >sP                 HH      «O 

S-H          1                             HH      ^                              |     I 

g  <£        g  S         g  8 

o 

g 

g 

TYPICAL 

a 

m 

H  S  ^      H    ?•            H   <» 

"2    .-3      2          *-"      I                  SH      J 

on  5    o       to    6            co    o 
O  ^    M       O   ^            O    ^ 

OH                          OH                          OH 

o  g         s  'S          ,82 

1 

CoS                  ^O,-,         ^'-'tn 

2 
O 

Q 

^Scn      ^'-S'i      R^^ 

W    ^A    ""^          W      P                  W     11     MH 
OSSr^         «o§         0^0n>< 
^t-iW        ^joO,       ycOC/J 

OH                         H                         U 

I 


SYSTEMATIC  DIAGNOSIS  83 

son  had  preserved  a  fair  ability  but  his  apathy  and  lack  of 
interest  interfered  with  tests,  and  possibly  also  with  the 
exercise  of  memory.  As  above  noted,  we  were  compelled 
to  maintain  the  suspicion  of  syphilis  throughout  despite 
the  attractive  hypotheses  of  traumatic  and  post-decom- 
pressive  effects  and  cerebral  tuberculosis.  A  history  of  the 
acquisition  of  syphilis  an  unknown  number  of  years  before 
admission  entered  to  strengthen  the  suspicion  of  the  syphilitic 
nature  of  the  mental  symptoms. 

The  W.  R.  proved  positive  in  blood  and  spinal  fluid.  The 
gold  sol  reaction  was  of  the  syphilitic  type;  37  cells  were 
found  per  cmm. ;  there  was  a  slight  amount  of  globulin  and  a 
slight  excess  of  albumin. 

We  made  a  diagnosis  of  CEREBROSPINAL  SYPHILIS  rather 
than  general  paresis  on  account  of,  first,  the  slow  course  of 
the  disease;  second,  the  vascular  type  of  the  cerebral  insult, 
hardly  typical  of  paresis;  and  third,  the  mild  spinal  fluid 
reaction.  Treatment  will  hardly  cure  the  hemiplegia,  at 
least  so  far  as  restoration  of  cerebral  tissues  lost  in  the  insult 
is  concerned.  We  were  perhaps  entitled  to  consider  that,  as 
in  the  cases  of  Petrofski  (17),  O'Neil  (19),  Robinson  (45), 
the  meningitic  process  could  be  arrested.  Unfortunately, 
our  treatment  of  20  injections  of  salvarsan  over  a  period 
of  10  weeks,  followed  by  a  number  of  months  of  bi-weekly 
injections  of  mercury  salicylate,  proved  incapable  of  making 
any  change  in  the  mental  and  physical  picture  or  in  the 
laboratory  findings. 

I.  Can  we  explain  the  apparently  poor  reaction  to  treatment  of 
the  cerebrospinal  syphilis  in  the  case  of  Jackson  by 
supposing  a  more  deep-seated  involvement  than  the 
meningovascular  involvement  indicated  by  the  hemi- 
plegia and  the  signs  in  the  fluid?  Autopsied  cases  in 
our  experience  show  focal  parenchymatous  involve- 
ments that  have  not  caused  obvious  clinical  symptoms 
at  any  time  during  the  course  of  the  disease.  These 
symptomatically  silent  lesions  may  have  been  present. 
'2.  What  is  the  comparative  prognostic  value  of  seizures 
in  paretic  neurosyphilis  and  in  such  a  meningovascular 
case  as  that  of  Jackson?  Paretic  seizures  are  often 
and  indeed  characteristically  recovered  from.  More- 


84  SYSTEMATIC  DIAGNOSIS 

over,  autopsies  in  paretic  neurosyphilis  characteris- 
tically show  no  gross  focal  destructive  lesions  to  cor- 
respond with  the  seizures.  The  paretic  seizures  are 
apparently  more  irritative  than  paralytic.  However, 
the  seizures  of  the  meningovascular  group  of  neuro- 
syphilis are  also,  though  less  commonly,  recovered  from, 
so  that  the  differential  diagnosis  on  the  basis  of  the 
outcome  of  seizures  is  not  safe.  Rarely  paretic  neuro- 
syphilis itself  also  develops  seizures  from  which  no 
recovery  is  made. 

3.  What  is  the  relation  of  neuropathic  heredity  to  neuro- 
syphilis? The  family  history  of  John  Jackson  is  un- 
doubtedly poor,  since  his  father  died  of  diabetes  and 
a  paternal  uncle  was  insane;  and  on  the  mother's  side, 
the  grandmother  died  of  tuberculosis  and  an  aunt  died 
insane.  This  general  question  was  more  interesting  in 
the  days  before  the  syphilitic  nature  of  general  paresis 
and  of  allied  diseases  was  known.  However,  we  may 
still  hold  perhaps  that  not  only  syphilis  but  also  vari- 
ous intoxications,  especially  alcoholism,  do  flourish  upon 
a  neuropathic  soil.  This  question,  like  that  of  Krafft- 
Ebing's  celebrated  claim  of  the  relation  between  syphil- 
ization  and  civilization,  needs  revision  in  the  light  of 
more  extensive  applications  of  the  W.  R.  in  larger 
and  larger  groups  of  persons  under  various  community 
conditions. 


SYSTEMATIC  DIAGNOSIS  85 


The  SIX  TESTS  (serum  Wassermann  reaction, 
fluid  Wassermann  reaction,  pleocytosis,  gold  sol 
reaction,  globulin,  excess  albumin)  are  likely  to 
run  STRONGER  in  PARETIC  NEUROSYPHILIS 
("  general  paresis  ")  than  hi  DIFFUSE  (especially 
meningovascular)  NEUROSYPHILIS;  hi  particu- 
lar, the  gold  sol  reaction  is  likely  to  prove 
"  paretic  "  rather  than  "  syphilitic."  The  clinical 
course  of  paretic  neurosyphilis  ("  general  paresis  ") 
is  likely  to  terminate  hi  death  within  a  few 
years. 


Case  15.  Pietro  Martiro  was  a  well-developed  and  nour- 
ished man,  30  years  of  age,  who  had  been  doing  erratic  things 
and  acting  peculiarly  for  a  few  weeks  before  entering  the  hos- 
pital. In  the  hospital,  Martiro  proved  to  be  very  excitable 
and  given  to  violence.  He  had  marked  delusions  of  grandeur, 
saying  he  was  worth  many  millions  of  dollars,  was  the  greatest 
singer  in  the  world,  the  greatest  athlete  in  the  world,  and 
the  like. 

Physically,  there  was  no  disorder  except  overactivity 
of  some  reflexes.  The  diagnosis  of  GENERAL  PARESIS  offered 
no  difficulties,  and  it  was  confirmed  by  the  laboratory  tests 
(positive  serum  and  fluid  W.  R.,  "  paretic  "  gold  sol  reaction, 
42  cells  per  cmm.,  an  excess  of  albumin,  and  a  positive 
globulin  test) . 

Treatment:  The  perfect  physique  of  this  case  and  the 
extremely  brief  clinical  duration  (a  few  weeks)  would  naturally 
suggest  a  probably  favorable  outcome.  However,  cases 
with  marked  delusions  of  grandeur  have  very  frequently 
proved  to  be  cases  with  extensive  brain  tissue  loss  as  shown 
in  certain  studies  with  Danvers  material. 

In  any  event,  the  treatment  in  this  case  proved  unavailing. 
Enormous  doses  of  salvarsan,  twice  a  week,  aided  by  mercury 
and  potassium  iodid,  were  given.  Although  other  cases  had 
been  helped  by  such  intensive  treatment,  Martiro  went 


86  SYSTEMATIC   DIAGNOSIS 


PARETIC  NEUROSYPHILIS  (GENERAL  PARESIS) 

PHYSICAL  SYMPTOMS 

EARLY  HEADACHE 
VISUAL  DISORDER 
HYPALGESIA 
ADIADOCHOKINESIS 
ATAXIA 

NASOLABIAL  FLATTENING 

VOCAL  CHANGE 

SPEECH  DISORDER: 

WRITING  DISORDER 

LOSS  OF  MANUAL  DEXTERITY 

PUPILLARY  CHANGES 

REFLEX  CHANGES 

SEIZURES 

LATE:  PARALYSIS,  CONTRACTURE 


CHART  9 


SYSTEMATIC  DIAGNOSIS  87 


PARETIC  NEUROSYPHILIS  (GENERAL  PARESIS) 

MENTAL  SYMPTOMS 

INTAKE  IMPAIRED 
CONSCIOUSNESS  CLOUDED 
FATIGUABILITY  INCREASED 
HALLUCINOSIS  RARE 

AMNESIA  —  RECENT!    CHRONOLOGY  AND  STORAGE 
IMPAIRED.    FABULATION 

OVERSUGGESTIBILITY 

JUDGMENT  IMPAIRED 

FANTASTIC  DELUSIONS 

INSIGHT  INTO  ILLNESS  NIL 

EARLY  IRRITABILITY  OR  HEBETUDE 

QUICK  SHIFTING  EMOTION 

CHARACTER  CHANGE 

CONDUCT  SLUMP 


CHART  10 


gg  SYSTEMATIC  DIAGNOSIS 

steadily  downhill,  nor  was  there  the  slightest  diminution  in 
the  intensity  of  any  of  the  spinal  fluid  reactions.  After  50 
injections  of  salvarsan  over  a  period  of  30  weeks  without  im- 
provement, treatment  was  discontinued.  A  few  months 
later,  the  patient  died. 

1.  What  is  the  duration  of  paretic  neurosyphilis  ("  general 

paresis")?  If  we  omit  the  doubtful,  early,  and  pro- 
dromal stages  and  count  the  beginning  of  the  disease 
with  the  occurrence  of  definite  symptoms,  we  find 
(Kraepelin)  that  almost  half  the  patients  with  pro- 
nounced paretic  signs  die  within  the  first  two  years  of 
their  disease.  Kraepelin's  observations  upon  244  cases 
are  as  follows: 

Year: I      2      3      4      56789    10    14 

Cases: 51     63    52    41     22    4    5    2    2       I       I 

The  average  duration  of  the  disease  in  months  has 
been  calculated  as  varying  from  24  to  32  months. 
Juvenile  paresis  runs  a  slower  and  more  insidious 
course.  The  duration  of  paresis,  according  to  many 
observers,  diminishes  with  the  increasing  age  of  the 
patient.  It  is  now  held  that  a  combination  of  tabes 
with  paresis  does  not  prolong  the  duration  of  the 
paresis.  As  noted  above  in  the  discussion  of  Case 
Harrison  (9),  our  conceptions  of  the  characteristic 
duration  of  paretic  neurosyphilis  must  alter  with  the 
increase  of  our  knowledge  due  to  the  early  application 
of  laboratory  tests. 

2.  What  is  the  significance  of  the  term  general  paresis? 

The  case  of  Martiro  is,  of  course,  a  good  instance  to 
show  that  the  term  is  sometimes  a  misnomer.  The 
characteristic  generalized  motor  incapacity  denoted 
by  the  term  general  paresis  is  shown  in  patients  in  the 
institutions  for  the  chronic  insane  in  their  last  few 
months  of  life.  The  term  paresis  is  perhaps  to  be 
preferred  to  the  term  paralysis  because  the  paralysis  is 
not  complete  but  partial;  but  perhaps  the  best  reason 
is  that  the  word  paresis  is  a  shorter  word.  When  the 
mental  side  is  to  be  emphasized,  the  term  paralytic 
dementia  is  employed.  In  this  book  we  have  used  the 
term  paretic  neurosyphilis  to  mean  a  more  precise 
statement  of  the  etiology  of  general  paresis  (general 
paralysis,  paralytic  dementia).  The  lay  term,  softening 


Euphoria  in  paretic  neurosyphilis  ("general  paresis").  The  head, 
arms  and  trunk  were  shaking  with  mirth;  hence,  the  indistinct  out- 
lines of  the  photograph. 


SYSTEMATIC   DIAGNOSIS  89 

of  the  brain,  like  the  terms  metasyphilis  and  parasyphilis 
is  in  the  present  phase  of  our  knowledge  to  be  eschewed. 
If  this  fatal  case  be  typical  of  general  paresis  (for  more 
favorable  results,  see  Part  V),  what  is  the  toll  of  deaths 
from  this  disease  in  the  community  at  large?  A  striking 
statement  may  be  quoted  from  Dr.  Thomas  W.  Salmon's 
"  Analysis  of  General  Paralysis  as  a  Public  Health 
Problem:" 

"  With  the  information  in  our  possession  at  the 
present  time,  we  are  able  to  state  that  not  fewer  than 
1000  persons  in  whom  general  paralysis  is  recognized 
die  in  New  York  State  every  year.  Let  us  compare 
this  with  the  lives  lost  from  some  other  important 
preventable  diseases.  It  means  that  one  in  nine  of 
the  6909  men  who  died  between  the  ages  of  40  and  60 
in  New  York  last  year  died  from  recognized  general 
paralysis  and  that  one  in  thirty  of  the  5299  women 
who  died  in  the  same  age-period  died  from  this  disease. 
'  The  number  of  deaths  from  general  paralysis  in 
New  York  last  year  about  equalled  the  number  of 
deaths  from  typhoid  fever.  The  following  table  gives 
the  number  of  deaths  due  to  the  ten  most  impor- 
tant specific  infectious  diseases.  Of  course,  deaths  in 
measles,  typhoid  fever  and  scarlet  fever  will  be  found 
also  under  the  names  of  some  of  the  complications  of 
these  diseases,  but  it  should  be  remembered  that 
these  primary  diseases  are  not  invariably  fatal  as  general 
paralysis  is.  Many  of  the  patients  with  measles  who 
died  from  bronchopneumonia  would  have  recovered 
but  for  this  complication,  while  the  paretics  with 
bronchopneumonia  would  have  died  even  if  this  com- 
plication had  not  arisen.  No  attempt  is  being  made  to 
compare  the  prevalence  of  general  paralysis  with  that 
of  other  diseases  —  we  are  trying  only  to  estimate  its 
share  in  the  mortality. 

"  I.   Tuberculosis  (all  forms) 16,133 

2.  Pneumonia 9,302 

3.  Bronchopneumonia 7>2I7 

4.  Diphtheria  and  croup 1,854 

5.  Influenza 1,381 

6.  Measles 1,071 

7.  Typhoid  Fever 1,018 

General  paralysis  (recognized} . .  1,000 

8.  Scarlet  fever 837 

9.  Whooping  cough 818 

10.   Syphilis 782  " 


00  SYSTEMATIC  DIAGNOSIS 


PARETIC  NEUROSYPHILIS  (GENERAL  PARESIS) 

CHARACTERISTICS 

AMNESIA 

QUICK  SHIFTING  EMOTIONS 

CHARACTER  CHANGE 

CONDUCT  SLUMP 

NERVOUS  DISORDERS 

SPEECH  DISORDERS 

PUPILLARY  CHANGES 

REFLEX  CHANGES 

SEIZURES 

CEREBROSPINAL  FLUID  PICTURE 


CHART  n 


SYSTEMATIC  DIAGNOSIS  9 1 


SYPHILITIC  PSYCHOSES 

SYPHILITIC  NEURASTHENIA 

GUMMA 

SYPHILITIC  PSEUDOPARESIS 

APOPLECTIC  CEREBRAL  SYPHILIS 

SYPHILITIC  EPILEPSY 

SYPHILITIC  PARANOIA 

TABETIC  PSYCHOSIS 

HEREDITARY 

PARESIS 


KRAEPELIN,  1910 


CHART  12 


92  SYSTEMATIC  DIAGNOSIS 


TABOPARETIC  NEUROSYPHILIS  ("  tabopare- 
sis ")  is  CLINICALLY  a  combination  of  the 
symptoms  of  TABES  DORSALIS  and  those  of 
GENERAL  PARESIS.  The  COURSE  of  TABO- 
PARESIS  is  likely  to  be  from  a  characteristic 
tabes  dorsalis  (often  of  years'  standing)  to  a  char- 
acteristic general  paresis;  the  ultimate  paretic 
picture  is  likely  to  retain  various  characteristics 
of  tabes.  The  LABORATORY  TESTS  in  the 
paretic  phase  are  characteristic  of  ordinary  (non- 
tabetic)  general  paresis.  The  PROGNOSIS, 
after  the  paretic  phase  has  arrived,  is  apt  to  be 
that  of  general  paresis. 


Case  16.  Joseph  Sullivan,  a  waiter,  50  years  of  age,  sought 
assistance  at  the  Psychopathic  Hospital  voluntarily.  His 
complaint  of  severe  and  lancinating  pains  in  the  legs,  difficulty 
with  his  gait,  and  a  feeling  of  constriction  about  the  waist, 
was  forthwith  suggestive  of  tabes  dorsalis.  He  was  a  rather 
poorly  nourished,  white-haired  man,  with  a  drooping  of  the 
left  side  of  the  face.  The  pupils  reacted  sluggishly  to  light, 
the  right  somewhat  better  than  the  left.  A  marked  Romberg 
reaction  could  be  demonstrated.  Ataxia  in  walking  was 
marked.  There  was  some  incoordination  of  the  hands,  con- 
siderable tremor,  and  writing  was  poorly  performed.  The 
ankle-jerks  and  knee-jerks  were  absent.  On  the  whole, 
the  diagnosis  of  TABES  DORSALIS  was  clear  enough. 

The  most  appealing  situation  was  mental.  Sullivan  was 
exceedingly  apprehensive  about  his  condition  on  the  ground 
that  it  was  growing  progressively  worse;  if  it  was  to  get 
worse,  Sullivan  feared  he  would  commit  suicide.  From  his 
own  account,  he  had  become  irritable,  quick-tempered,  and 
often  unreasonable.  As  usual  in  these  cases,  the  question 
arose  whether  the  depression  was  psychopathic  or  natural. 

While  in  the  hospital  things  shortly  came  to  a  crisis.  In 
the  midst  of  a  fit  of  depression,  Sullivan  attempted  suicide 


SYSTEMATIC  DIAGNOSIS  93 

TABETIC  SYMPTOMS  AND  SIGNS  IN  ORDER 
OF  THEIR  FREQUENCY 

ANALYSIS  OF  250  CASES 

PER  CENT 

1.  ROMBERG  SIGN 96.4 

2.  ABSENT  KNEE-JERKS 90.0 

3.  LANCINATING  PAINS 88.4 

4.  STAGGERING  GAIT 87.2 

5.  ARGYLL-ROBERTSON  PUPIL 80.0 

6.  ATAXIA  IN  UPPER   EXTREMITIES 68.2 

7.  SPHINCTER  DISTURBANCES 67.6 

8.  SENSORY  DISTURBANCES 58.2 

9.  VISUAL  DISTURBANCES 43.6 

10.  PARESTHESIA  AND  NUMBNESS  OF  FEET    AND 

LOWER  EXTREMITIES 42.8 

11.  GIRDLE  SENSE 31.2 

12.  PTOSIS  OF]  EYE-LIDS 23.2 

13.  PARESTHESIA     OR     NUMBNESS     IN  HANDS  OR 

UPPER  EXTREMITIES 13.6 

14.  STRABISMUS 12.0 

15.  VISCERAL  CRISES 12.0 

16.  LOSS  OF  SEXUAL   DESIRE 11.5 

17.  CHARCOT  JOINTS 9.2 

18.  VERTIGO 4.0 

19.  MAL  PERFORANS 3.2 

20.  PAIN  IN  JOINTS 2.8 

21.  RECTAL  TENESMUS 2.8 

22.  MENTAL  DEGENERATION   (other  than  paresis) 2.4 

23.  HEMIPLEGIA 2.4 

24.  VESICAL  TENESMUS 2.0 

25.  DIFFICULTY  IN  ARTICULATION 2.0 

26.  DEAFNESS 1.2 

27.  ANOSMIA 0.8 

BALDWIN  LUCKE. 
CHART  13 


94 


SYSTEMATIC  DIAGNOSIS 


by  beating  his  head  against  the  wall.  Whether  this  attempt 
could  be  regarded  psychopathic,  however,  remained  in  ques- 
tion. Sullivan  had  been  drinking  very  heavily  although  he 
had  stopped  about  six  weeks  before  admission,  fearing  that 
the  alcohol  was  causing  a  development  of  symptoms.  The 
remedy  was  almost  worse  than  the  disease  because  he  then 
became  more  nervous,  lost  his  appetite,  and  had  a  marked 
insomnia. 

According  to  the  patient's  own  history,  he  had  had  several 
attacks  of  gonorrhoea  and  a  syphilitic  infection  at  the  age  of 
19;  that  is,  some  31  years  before  admission  to  the  hospital. 
However,  the  first  neurological  symptoms  of  which  the  patient 
was  aware  came  about  27  or  28  years  after  infection,  namely, 
3  or  4  years  before  admission,  when  facial  paralysis  developed. 
At  that  time,  he  had  suddenly  felt  a  peculiar  sensation  in  the 
throat  and  became  unable  to  swallow  for  a  time.  His  voice 
remained  hoarse  and  low  for  some  time,  and  his  face  began  to 
droop.  The  lancinating  pains  and  the  ataxia  also  dated 
back  several  years. 

1.  How  shall  we  evaluate  the  mental  symptoms?  The  prog- 

nosis of  tabes  dorsalis  is  relatively  good  so  far  as  life  is 
concerned,  and  it  might  even  be  possible  for  Sullivan  by 
training  to  remain  capable  of  being  a  waiter.  The 
manual  incoordination  was  not  marked,  and  possibly 
the  manual  tremor  was  in  part  due  to  alcohol.  Accord- 
ingly, the  mental  symptoms,  such  as  emotional  lability 
and  memory  defect,  were  in  the  foreground  of  attention. 
In  point  of  fact,  the  laboratory  examinations  showed 
positive  W.  R.  in  the  serum  and  the  spinal  fluid,  which 
latter  also  contained  60  cells  per  cmm.,  positive  glob- 
ulin, and  an  excess  of  albumin.  THE  DIAGNOSIS  MADE 
WAS  THAT  OF  TABOPAREsis,  meaning  thereby  a  tabes 
associated  with  appropriate  symptoms  of  a  mental 
nature. 

2.  How  shall  the  term  taboparesis  be  used?    Some  use  the 

term,  as  we  feel  erroneously,  for  instances  of  general 
paresis  which  happen  to  show  crural  areflexia  (absence 
of  knee-jerks).  We  feel  that  the  best  usage  of  the 
term  is  for  instances  in  which  well-defined  symptoms  of 
tabes  (as  well  as  of  paresis)  are  present,  namely,  char- 
acteristic ataxia,  lightning  pains,  and  the  like.  If  the 


SYSTEMATIC  DIAGNOSIS  95 

term  is  used  more  loosely,  as  above  mentioned,  then 
practically  every  case  of  general  paresis  might  perhaps 
be  termed  taboparesis,  since  almost  every  case  of  paresis 
does  show  involvement  of  the  cord  as  well  as  of  the 
cerebrum.  Such  involvement  may  lead  to  hyperre- 
flexia,  hyporeflexia,  or  areflexia  according  to  the  localiza- 
tion of  the  process.  In  true  taboparesis,  in  which  there 
is  a  commingling  of  the  features  of  tabes  with  those  of 
paresis,  we  should  find  the  posterior  roots  of  the  spinal 
cord  affected.  The  spinal  lesions  of  paresis  itself  are 
more  apt  to  be  intraspinal;  that  is,  confined  to  the 
nervous  system  within  the  pial  investment. 

Bearing  in  mind  that  Sullivan  was  a  waiter,  what  shall 
be  said  about  the  infectivity  of  these  cases?  It  is 
counted  as  a  rule  as  negative,  since  there  are  no  open 
spirochete-bearing  lesions.  The  longer  the  period  since 
infection  the  less,  as  a  rule,  is  the  chance  of  contagion 
in  syphilis ;  and  as  tabes  and  paresis  occur  fairly  late  in 
the  disease,  the  infectiousness  at  this  stage  is  practically 
negligible. 

Of  what  differential  value  is  the  insight  shown  by  Sulli- 
van into  the  nature  of  his  symptoms?  Kraepelin 
remarks  that  a  genuine  insight  into  the  nature  of  the 
disease  does  not  as  a  rule  occur  in  paresis.  At  the 
beginning  of  the  disease,  there  may  sometimes  be  a 
correct  understanding  of  the  nature  of  the  disease  and 
of  its  probable  outcome ;  but  the  presence  or  absence  of 
insight  into  the  fact  of  mental  disease  is  by  no  means  a 
differential  sign  of  practical  value. 

What  is  to  be  said  of  the  occurrence  of  depression  and 
excited  states  in  paretic  neurosyphilis?  A  variety  of 
classifications  of  sub-forms  of  paretic  neurosyphilis 
have  been  propounded.  Kraepelin,  for  example,  deals 
with  four:  the  demented,  depressive,  expansive,  and 
agitated  forms,  but  remarks  that  the  division  is  merely 
convenient  for  exposition.  The  institutional  intake 
does  not  accurately  represent  the  distribution  of  cases. 
Under  psychopathic  hospital  conditions  with  the  rela- 
tively easy  resort  to  such  institutions,  the  number  of 
quiet  cases  increases;  under  the  less  advanced  condi- 
tions in  Heidelberg,  Kraepelin  took  in  53%  demented 
paretics  as  against  56%  at  Munich  (73%  women^  under 
the  easier  conditions  of  admission.  The  admissions  ^of 
demented  paretics  varied  from  37  to  56%.  The  varia- 
tions depend  much  upon  the  facility  with  which  the 
cases  can  be  brought  to  institutions.  Where  admission 


96  SYSTEMATIC  DIAGNOSIS 

is  beset  with  various  legal  restrictions,  the  quiet  and 
demented  cases  are  more  apt  to  be  treated  for  long 
periods  at  home.  The  depressive  type  of  paretic 
neurosyphilis  forms  a  much  smaller  group,  according 
to  Kraepelin,  as  only  about  12%  of  his  Heidelberg  ad- 
missions were  of  this  type,  and  still  fewer  of  his  Munich 
admissions.  Other  authors  give  percentages  as  high  as 
1 6  and  19.  The  so-called  expansive  group  is  larger, 
Kraepelin  finding  30%  of  his  Heidelberg  cases  to  be  of 
this  group,  and  21  to  22%  of  his  Munich  cases.  The 
rarest  sub-form  of  paretic  neurosyphilis  is  the  agitated 
form:  6%  of  Kraepelin's  Heidelberg  admissions;  14% 
among  males  and  5%  among  females  in  his  Munich 
admissions,  where  the  diagnosis  of  agitated  paresis  was 
entered  on  somewhat  broader  lines.  French  authors 
(Serieux  and  Ducaste)  have  enlarged  the  number  of 
sub-forms  of  paretic  neurosyphilis  as  follows :  Expansive 
27% ;  sensory  24% ;  demented  24% ;  persecutory  3% ; 
depressive  2% ;  circular  7% ;  hypochondriacal  7% ;  and 
maniacal  6%. 


SYSTEMATIC  DIAGNOSIS  97 


DIFFUSE  (meningovasculoparenchymatous)  NEU- 
ROSYPHILIS  may  look  precisely  like  PARETIC 
NEUROSYPHILIS  ("  general  paresis  ")  at  certain 
periods  of  clinical  and  laboratory  examination. 


Case  17.  The  police  found  Gregorian  Petrofski  crouching 
on  his  knees  on  a  Boston  sidewalk,  attempting  to  take 
pickets  off  a  fence.  Petrofski  knew  little  English;  he  said 
that  he  had  slept  in  Poland  the  night  before.  He  did  not 
appear  to  be  alcoholic. 

When  he  was  examined,  through  an  interpreter,  he  told 
how  he  had  been  in  America  two  days,  and  in  Boston  two 
years;  that  he  was  at  the  present  time  in  Poland,  and  that 
his  brother  had  brought  him  to  the  hospital  and  left  him 
there. 

The  physical  examination  showed  Petrofski  to  be  well 
developed  and  nourished.  His  pupils  were  somewhat  dilated 
and  reacted  somewhat  slowly  to  light  and  accommodation. 
Neurologically,  there  was  nothing  else  abnormal  found  upon 
systematic  examination  although,  through  lack  of  cooperation, 
sensory  and  coordination  tests  proved  difficult  if  not  impos- 
sible. There  was  a  large  ulcer  on  the  under  surface  of  the 
glans  penis,  with  several  small  smooth  scars  on  the  upper 
surface.  There  was  a  purulent  discharge  from  the  external 
meatus.  There  were  exostoses  of  both  tibiae. 

The  initial  diagnosis  had  to  consider  uremia  and  diabetes, 
which  could  be  easily  excluded  on  examination.  Alcoholism 
was  excluded  through  absence  of  alcohol  on  the  breath. 
There  remained  such  diagnoses  as  epilepsy,  some  post-trau- 
matic condition,  or  meningitis,  to  say  nothing  of  the  hypoth- 
esis of  syphilis  raised  by  the  tibial  exostoses  and  the  lesions 
of  the  penis.  The  hypothesis  of  trauma  was  given  up,  as 
well  as  epilepsy  and  meningitis  upon  the  data  of  the  lumbar 
puncture.  The  spinal  fluid  proved  to  be  clear  but  with 
enormous  amounts  of  globulin  and  albumin,  80  cells  per  cmm., 
a  "  paretic  "  gold  sol  reaction,  and  a  positive  spinal  fluid 


C$  SYSTEMATIC  DIAGNOSIS 

W.  R.  (the  serum  W.  R.  was  also  positive).  Accordingly,  it 
was  clear  that  the  case  was  one  of  neurosyphilis. 

Treatment  was  instituted  with  injections  of  mercury  sal- 
icylate,  a  grain  and  a  half  twice  a  week,  and  potassium  iodid. 
After  some  weeks,  diarrhoea  and  salivation  with  marked 
symptoms  of  mercury  poisoning  set  in;  the  treatment  was 
suspended,  but  later  re-instituted.  In  a  few  weeks  Petrofski 
was  apparently  quite  well,  the  spinal  fluid  tests  had  all  become 
negative,  as  had  the  serum  W.  R. 

Petrofski  now  began  to  pick  up  a  good  deal  of  English,  and 
gave  a  consistent  narrative  of  his  past  life,  although  the 
period  just  prior  to  and  during  his  early  stay  in  the  hospital 
has  remained  blank.  Without  further  treatment  Petrofski 
has  remained  well  for  over  a  year. 

I.  Does  the  "  paretic  "  gold  sol  reaction  mean  general  paresis? 
In  connection  with  this  general  question,  a  brief  sum- 
mary of  the  significance  of  the  gold  sol  reaction  in  this 
group  may  be  made,  (i)  Fluids  from  cases  of  general 
paresis  in  the  vast  majority  of  cases  will  give  a  strong 
and  fairly  characteristic  reaction,  especially  if  more 
than  one  sample  is  tested.  (2)  Very  rarely  general 
paresis  fluid  will  give  a  reaction  weaker  than  the  char- 
acteristic one.  (3)  Fluids  from  cases  of  syphilitic 
involvement  of  the  central  nervous  system  other  than 
general  paresis  often  give  a  weaker  reaction  than  the 
paretic,  but  in  a  fairly  high  percentage  of  cases  give 
the  same  reaction  as  the  paretics.  (4)  Non-syphilitic 
cases  may  give  the  same  reaction  as  the  paretics ;  these 
cases  are  usually  chronic  inflammatory  conditions  of 
the  central  nervous  system.  (5)  When  a  syphilitic 
fluid  does  not  give  the  strong  "paretic  reaction"  it  is 
presumptive  evidence  that  the  case  is  not  general  paresis, 
and  this  test  offers  a  very  valuable  differential  diagnostic 
aid  between  general  paresis,  tabes,  and  cerebrospinal 
syphilis.  (6)  The  term  "  syphilitic  zone  "  is  a  mis- 
nomer, as^  non-syphilitic  as  well  as  syphilitic  cases  give 
reactions  in  this  zone,  but  no  fluid  of  a  case  with  syphi- 
litic central  nervous  system  disease  has  given  a  reaction 
out  of  this  zone,  so  that  the  finding  may  be  used  nega- 
tively; and  any  fluid  giving  a  reaction  outside  of  this 
zone  may  be  considered  non-syphilitic.  (7)  Mild  re- 
actions may  occur  without  any  evident  significance, 


SYSTEMATIC  DIAGNOSIS  99 


FREQUENT  SYMPTOMS  IN  DIFFUSE  AND 
VASCULAR  NEUROSYPHILIS 

("  CEREBRAL  »  AND   "  CEREBROSPINAL  SYPHILIS  ») 

PUPILLARY  DISORDER 

HEADACHE 

VERTIGO 

INSOMNIA 

DROWSINESS 

CHANGE  IN  DISPOSITION 

Irritability        Slow  thinking 

SEIZURES 

PARALYSES 

Permanent        Transient 

APHASIA 

HEMIANOPSIA 

SENSORY  DISTURBANCES 

GASTRIC  CRISES 

SPHINCTER  DISTURBANCES 

INTRACRANIAL  PRESSURE  SYMPTOMS 

POLYURIA,  POLYDYPSIA,  GLYCOSURIA 

MENIERE'S  SYNDROME 

NYSTAGMUS 

CHART  14 


IOO  SYSTEMATIC   DIAGNOSIS 

while  a  reaction  of  no  greater  strength  may  mean  marked 
inflammatory  reaction.  (8)  Tuberculous  meningitis, 
brain  tumor,  and  purulent  meningitis  fluids  character- 
istically, though  not  invariably,  give  reactions  in  higher 
dilutions  than  syphilitic  fluids.  (9)  The  unsupple- 
mented  gold  sol  test  is  insufficient  evidence  on  which  to 
make  any  diagnosis,  but  used  in  conjunction  with  the 
W.  R.,  chemical  and  cytological  examinations,  it  offers 
much  information,  aiding  in  the  differential  diagnosis 
of  general  paresis,  cerebrospinal  syphilis,  tabes  dorsalis, 
brain  tumor,  tuberculous  meningitis,  and  purulent  men- 
ingitis. (10)  We  believe  that  no  cerebrospinal  fluid 
examination  is  complete  for  clinical  purposes  without 
the  gold  sol  test. 

See  Appendix  B  for  technical  details. 

2.  What  is  the  relation  of  the  tibial  exostosis  to  neuro- 
syphilis?  The  syphilographers  have  always  stressed 
the  tibial  lesions  in  the  diagnosis  of  syphilis.  Although 
not  so  much  attention  has  been  paid  to  these  and 
kindred  osseous  lesions  in  neurosyphilis,  yet  we  have 
frequently  found  such  lesions  and  they  afford  an  im- 
portant auxiliary  means  of  diagnosis. 


SYSTEMATIC  DIAGNOSIS  IOI 


A  POSITIVE  SERUM  Wassermann  reaction  with 
a  NEGATIVE  FLUID  Wassermann  Reaction  may 
be  found  in  NEURO SYPHILIS,  particularly  in 
VASCULAR  NEUROSYPHILIS :  the  remaining 
signs  in  the  fluid,  although  frequently  positive,  may 
even  be  negative. 


Case  1 8.  Frederick  Wescott  was  a  promoter,  an  elderly 
looking  man  of  60  years.  His  health  had  been  failing  for  18 
months.  There  had  been  shortness  of  breath,  dizziness,  a 
tired  feeling,  inability  to  "  get  the  words  he  wanted,"  and 
forgetfulness  of  names.  About  eight  weeks  before  examina- 
tion, Wescott  had  had  a  convulsion,  following  which  he  had 
been  unable  to  express  himself  at  all  well.  This  convulsion 
was  not  accompanied  by  loss  of  consciousness.  Besides  a 
marked  motor  aphasia,  there  was  agraphia. 

Physically,  Wescott  showed  arteriosclerosis  and  a  blood 
pressure  of  135  systolic,  but,  except  very  lively  knee-jerks, 
no  other  reflex  disorders  or  anomalies  were  discovered.  In 
particular,  the  pupils  reacted  fairly  well. 

There  was,  perhaps,  no  special  reason  to  implicate  syphilis 
in  the  case,  yet  Wescott  gave  a  history  of  syphilis  at  35  years. 
The  W.  R.  of  the  blood  serum  proved  positive;  that  of  the 
spinal  fluid  was  negative,  and  the  albumin  was  but  slightly 
increased;  there  was  a  very  slight  amount  of  globulin,  and 
there  were  16  cells  per  cmm.  in  the  fluid.  The  gold  sol  re- 
action suggested  syphilis. 

We  felt  entitled  to  make  a  diagnosis  of  SYPHILITIC  CERE- 
BRAL ARTERIOSCLEROSIS,  regarding  the  convulsion  or  seizure 
eight  weeks  before  as  due  to  a  vascular  insult.  The  labora- 
tory picture  in  the  spinal  fluid  in  Wescott's  case  seems  to  be 
rather  characteristic  of  this  group  of  syphilitic  arteriosclerotics. 

I.  What  is  the  reason  for  the  negative  spinal  fluid  W.  R.? 
The  theory  would  be  that  the  syphilitic  lesion  is  local- 
ized in  the  vascular  system  and  that  the  parenchyma 


IO2  SYSTEMATIC  DIAGNOSIS 

is  only  secondarily,  if  at  all,  involved.  The  W.  R. 
producing  bodies  are  accordingly  not  found  in  the  fluid. 

2.  How  frequently  are  several  of  the  spinal  fluid  tests  neg- 

ative, while  others  are  positive?  Whereas,  clinically 
speaking,  the  five  tests  in  the  spinal  fluid  (W.  R.,  globulin 
reaction,  excess  albumin,  pleocytosis,  and  gold  sol 
reaction)  are  each  indicative  of  a  pathological  con- 
dition in  the  central  nervous  system,  yet  a  specially 
intensive  study  of  the  distribution  of  these  tests  has 
shown  that  they  are  prone  to  occur  independently. 
Consequently,  we  must  concede  that  they  do  not  all 
represent  the  same  inflammatory  products  and  chemical 
conditions.  The  W.  R.  producing  bodies,  the  gold  sol 
reaction  producing  bodies,  as  well  as  the  globulins  and 
albumins,  have  been  proved  to  be  separate.  Special 
work  has  also  shown  that  these  tests  disappear  under 
treatment  at  different  rates.  There  is,  unfortunately,  no 
doubt  that  the  rate  and  intensity,  presence  or  absence, 
and  the  order  of  disappearance  of  these  tests  in  either 
treated  or  untreated  cases,  do  not  at  all  parallel  the 
clinical  conditions  of  the  patients. 

3.  What  is  the  prognosis  in  vascular  neurosyphilis,  such  as 

in  the  case  of  Wescott?  The  prognosis  is  identical 
with  that  of  cerebral  arteriosclerosis  in  general,  that 
is  to  say,  bad,  but  with  frequent  periods  of  improve- 
ment. In  the  neurosyphilitic  type  of  arterial  disease 
thromboid  formation  is  frequent.  Where  the  lesion  is 
chiefly  pervascular  infiltration,  rather  than  disintegra- 
tion of  the  vessel  wall,  improvement  may  very  well 
occur  as  a  result  of  treatment.  Wescott  showed  slight 
improvement  under  treatment.  He  has  already  lived 
two  years  since  his  first  convulsion,  and  three  and  a 
half  years  since  the  onset  of  symptoms 


SYSTEMATIC  DIAGNOSIS  103 


DIFFUSE    NEUROSYPHILIS    (so-called    "  cere- 
brospinal  syphilis  ")  is  often  marked  by  SEIZURES. 


Case  19.  Agnes  O'Neil,  an  unmarried  woman  of  28  years, 
was  first  examined  five  weeks  after  the  initial  symptoms.  It 
appears  that  she  had  had  certain  seizures,  with  unconscious- 
ness and  twitching  of  the  limbs  (otherwise  not  well  described), 
followed  by  confusion  of  mind  and  sometimes  by  a  weakness 
of  the  left  side  and  a  difficulty  in  speaking.  Headache  had 
been  almost  constant,  as  well  as  pains  in  the  arms  and  legs. 

Physically,  both  in  general  and  neurologically,  there  were 
no  signs  or  symptoms ;  mentally,  we  could  discover  no  symp- 
toms. Syphilis  was  denied,  although  possible  exposure  to 
syphilis  was  admitted. 

The  diagnosis  of  some  form  of  organic  brain  disease  was 
clear  with  the  picture  of  convulsions  followed  by  slight  aphasia 
with  headaches  and  limb  pains.  With  onset  at  28,  the  most 
frequent  cause  for  such  epileptiform  seizures  is  certainly 
syphilis.  Examination  of  the  blood  and  spinal  fluid  showed 
a  positive  W.  R.,  in  both.  The  albumin  was  also  somewhat 
increased.  The  clinical  picture  suggested  a  fairly  generalized 
meningitic  involvement. 

The  prognosis  in  such  cases  of  generalized  meningitic 
involvement  is  in  general  good,  and  this  principle  was  illus- 
trated in  the  O'Neil  case,  in  which  the  symptoms  soon  dis- 
appeared under  intensive  antisyphilitic  treatment.  In  fact 
the  spinal  fluid  W.  R.  became  negative  in  the  course  of  four 
weeks.  The  blood  serum  W.  R.,  however,  has  remained 
positive  despite  eight  months  of  active  treatment. 

I.  Are  certain  cases  of  syphilitic  epilepsy  really  cases  of 
Jacksonian  epilepsy?  As  a  matter  of  nomenclature, 
Jacksonian  cortical  epilepsy  is  usually  the  result  of  a 
focal  and  circumscribed  irritative  lesion  in  the  cortex. 
Gumma,  local  syphilitic  meningitis,  and  syphilitic 
vascular  lesions,  as  well  as  scars  consequent  upon  the 
latter,  are  among  the  causes  of  Jacksonian  epilepsy, 


IO4  SYSTEMATIC  DIAGNOSIS 


CONDITIONS  IN  WHICH  CONVULSIONS  OCCUR 

I 

NEUROSYPHILIS 

HYSTERIA 

EPILEPSY  MAJOR  (GRAND  MAL) 

EPILEPSY  MINOR  (PETIT  MAL) 

DEMENTIA  PRAECOX 

TOXIC  CONDITIONS: 

Asphyxia,  Uremia,  Alcohol,  Absinthe,  Lead,  Mercury,  etc. 

ORGANIC  BRAIN  LESIONS 

Apoplexy,  Meningitis,  Intracranial  Growths 

STOKES-ADAMS  DISEASE 
MALINGERING 
DISSEMINATED  SCLEROSIS 


CHART  15 


SYSTEMATIC  DIAGNOSIS  105 

along  with  such  other  focal  lesions  as  trauma,  tumor 
abscess,  tubercle,  and  the  like.  Even  non-syphilitic 
Jacksonian  epilepsy  has  been  observed  from  time  to 
time  in  cases  of  diffuse  intracranial  pressure.  Jack- 
sonian attacks  also  have  been  found  in  so-called  genuine 
epilepsy.  Accordingly,  we  must  not  conclude  from  the 
occurrence  of  Jacksonian  convulsions,  even  though  in  a 
proved  syphilitic  case,  that  the  convulsions  in  ques- 
tion are  surely  due  to  a  focal  lesion,  for  they  may  be 
due  to  diffuse  syphilitic  lesions. 

2.  What  is  the  significance  of  aphasia  in  Agnes  O'Neil? 
Aphasia  is  not  a  characteristic  symptom  in  ordinary 
Jacksonian  epilepsy,  but  the  aphasia  is  another  sign  of 
focal  lesion  and  forms  an  added  argument  against  the 
diagnosis  of  genuine  or  idiopathic  epilepsy.  See  also 
discussion  of  aphasia  in  paretic  neurosyphilis  under 
Case  Levenson  (22). 

A.  What  is  the  behavior  of  the  serum  W.  R.  and  the  spinal 
fluid  W.  R.  under  systematic  treatment?  Sometimes, 
as  in  this  case,  the  serum  W.  R.  remains  positive  and  the 
fluid  W.  R.  becomes  negative;  but  in  other  equally 
well-defined  cases,  the  reverse  holds  true,  and  the  serum 
W.  R.  reaction  becomes  negative  whereas  the  spinal 
fluid  reaction  remains  positive.  The  obvious  conclu- 
sion is  that  we  cannot  always  be  sure  even  by  faithful 
tests  of  either  the  serum  or  the  fluid  alone,  whether  the 
treatment  has  succeeded  in  abolishing  the  laboratory 
signs. 

4.  Can  this  case  be  regarded  as  one  of  cure?  Not  by  the 
definition  adopted  in  this  book  or  by  the  syphilographers 
who  take  into  account  not  only  the  nervous  system  but 
the  body  which  contains  it.  To  be  sure,  the  spinal 
fluid  of  Agnes  O'Neil  is  now  entirely  negative  and  she  is 
clinically  free  from  symptoms;  yet  from  the  broad 
standpoint  of  syphilis  therapy  in  general,  this  patient  is 
not  cured,  as  is  evidenced  by  the  positive  serum  W.  R. 


I06  SYSTEMATIC   DIAGNOSIS 


PARETIC      NEUROSYPHILIS     ("  general 
paresis  ")  is  often  marked  by  SEIZURES. 


Case  20.  Lester  Crane,  a  plumber,  37  years  of  age,  came 
to  the  hospital  with  a  slow  and  defective  speech.  Moreover, 
there  seemed  to  be  some  mental  disorder  since  his  answers  to 
questions  were  not  always  relevant.  It  appeared  that  he 
was  seeing  bugs  on  the  wall. 

Physically,  Crane  was  a  well-developed  and  nourished  man, 
with  overactive  knee-jerks  and  a  Babinski  reaction  on  the 
left  side. 

It  developed  that  there  was  an  impairment  in  hearing. 
The  pupils  reacted  well  both  to  light  and  to  distance.  The 
patient  was  very  restless  and  smiled  in  a  silly  fashion.  His 
memory  was  decidedly  defective  in  all  spheres,  and  he  was 
very  slow  in  the  intake  of  ideas. 

The  plumber's  wife  said  that,  at  about  the  age  of  23  or  24, 
he  had  a  spell  of  confusion  lasting  two  or  three  days,  with 
peculiar  conduct,  unintelligible  talk,  and  a  good  deal  of 
weeping.  The  medical  diagnosis  at  that  time  took  into 
account  the  fact  that  Crane  was  a  plumber  and  was  "  lead 
encephalopathy." 

However,  according  to  his  wife,  Crane  had  acquired 
chancre  at  about  26  years,  was  treated  mercurially  for  about 
three  years  and  declared  well.  He  had  remained  well  up  to 
about  1 8  months  before  entrance,  when,  without  previous 
warning,  the  patient  had  a  convulsion  with  the  continuous 
movements  for  about  half  an  hour.  He  was  semi-conscious 
for  about  18  hours  and  vomited  continuously.  There  was 
amnesia  for  the  whole  affair  on  regaining  consciousness.  In 
a  week's  time,  Crane  was  entirely  well.  But  six  weeks  later 
there  was  another  convulsion.  Upon  removal  to  a  hospital, 
the  diagnosis  of  general  paresis  was  made,  and  the  patient 
was  given  the  Swift-Ellis  intraspinous  treatment.  This 
seemed  to  be  very  successful,  and  the  patient  discontinued 
treatment  after  14  weeks  (during  which  time  there  had 


SYSTEMATIC  DIAGNOSIS  IO7 

been  seven  treatments)  on  the  ground  that  he  was  entirely 
well. 

However,  after  discontinuing  treatment,  there  was  another 
convulsion  in  about  a  month,  and  further  convulsions  occurred 
once  a  month.  For  six  months,  however,  the  patient  took 
no  treatment,  but  finally  returned  to  the  hospital  and  was 
given  mercury.  This  treatment  appeared  to  suspend  con- 
vulsions again  for  three  months,  but  at  the  expiration  of 
six  months,  the  patient  had  three  convulsions  in  one  day, 
and  several  more  during  the  following  days.  After  the  last 
of  these  convulsions,  there  had  been  numbness  on  the  right 
side  of  the  body  and  considerable  headache. 

The  diagnosis  of  PARETIC  NEUROSYPHILIS  ("general  par- 
esis") is  borne  out  by  the  laboratory  tests.  The  W.  R.  of 
the  blood  serum  was,  to  be  sure,  negative,  but  the  W.  R.  of 
the  spinal  fluid  was  positive,  and  there  was  a  "  paretic  "  type 
of  gold  reaction,  together  with  other  laboratory  signs.  \ 

The  case  well  demonstrates  that  group  of  paretic  cases  in\ 
which  convulsions  periodically  occur,  leaving  the  patient  worse 
after  each  convulsion.     Treatment  with  salvarsan  was  insti- 
tuted, and  mercury  and  iodid  was  given  by  mouth.     During 
the  period  of  eight  months  which  have  now  elapsed  since  the 
beginning  of  this  treatment,  there  have  been  no  convulsions; 
there  has  been  a  great  improvement  in  the  memory,  the  hear-' 
ing  has  improved,  the  W.  R.  in  the  spinal  fluid  is  much  less 
intense,  the  gold  sol  test  has  become  negative,  and  the  other/ 
tests  are  all  less  intense.  / 

The  patient,  however,  has  not  been  entirely  well,  for  in 
place  of  the  generalized  convulsions,  he  has  had  minor  sei- 
zures, beginning  as  a  rule  with  a  tingling  sensation  in  the 
right  hand,  extending  up  the  arm,  down  the  trunk  and  leg,  and 
through  the  right  side  of  the  face,  with  a  bitter  sensation  on 
the  right  half  of  the  tongue.  The  patient  maintains  that 
this  sensation  is  absolutely  confined  to  the  right  half  of  the 
body  (in  this  connection  we  may  recall  case  Morton  (i),  in 
which  there  was  also  a  hemiplegia  together  with  other  ap- 
parently hysterical  symptoms  at  several  times  during  the 
long  course  of  a  disease  with  abundant  structural  correla- 
tions). During  these  minor  seizures,  the  patient  is  unable 


I0g  SYSTEMATIC  DIAGNOSIS 


LOSS  OF  DEEP  REFLEXES 

NEUROSYPHILIS 

NEURITIS 

(alcohol,  diabetes,  diphtheria,  lead,  arsenic,  tubercle,  cachexia,  etc.; 
Peripheral  nerves  sensory  or  motor 

PERIPHERAL  NERVE  PALSIES 

TEMPORARILY  FROM   COMPRESSION  BY  TURNIQUET 
FRIEDREICH'S  ATAXIA 

SUBACUTE  COMBINED  DEGENERATION  OF  POSTERIOR 
AND  LATERAL  COLUMNS 
Posterio'  column  disease  > 

FOCAL  LESION   IN  GRAY  MATTER  OF  CORD 

INFANTILE  PARALYSIS  (ACUTE  ANTERIOR  POLIOMYELITIS) 

PROGRESSIVE  MUSCULAR  ATROPHY 
(chronic  anterior  poliomyelitis) 
Anterior  cornua  of  cord 

AMYOTROPHIC  LATERAL  SCLEROSIS 

SYRINGOMYELIA 

THROMBOSIS  OF  ANTERIOR  SPINAL  ARTERY 

LANDRY'S  PARALYSIS 

Anterior  cornua  and  peripheral  motor  nerves 

MYOPATHIES 

(pseudohypertrophic  and  atrophic  types) 
Muscle  itself 

AMYOTONIA  CONGENITA 

FAMILY  PERIODIC  PARALYSIS 

(during  attacks) 

INCREASED  INTRACRANIAL  PRESSURE 

(especially  hydrocephalus  and  tumors  of  posterior  fossa) 

PNEUMONIA 

IMMEDIATELY  AFTER  ATTACK  OF  MAJOR  EPILEPSY 
(post-epileptic  coma) 

TOXIC  COMA 

(uremia,  morphine,  etc.) 

DURING  SPINAL  ANESTHESIA 

COMPLETE  TRANSVERSE  LESION  OF  CORD 

PURVES  STUART 

CHART  16 


SYSTEMATIC  DIAGNOSIS  ICK) 

to  talk,  although  he  does  not  lose  consciousness  and  is  en- 
tirely aware  of  everything  going  on  about  him.  These 
attacks  have  of  late  been  growing  somewhat  less  frequent. 

1.  What  is  the  cause  of  the  negative  serum  W.  R.?     It  is 

claimed  that  3  to  5%  of  all  cases  of  general  paresis 
yield  a  negative  blood  serum.  In  this  particular  case, 
there  had  been  considerable  treatment,  including  some 
Swift-Ellis  treatment,  so  that  it  may  be  that  this 
treatment  had  reduced  a  formerly  positive  blood  serum 
W.  R.  to  a  negative  one. 

2.  What  is  the  nature  of  the  typical  seizures  of  general 

paresis?  The  most  frequent  seizures  are  epileptiform 
and  bear  a  general  resemblance  to  cortical  epilepsy; 
but  more  rarely  these  seizures  resemble  the  ordinary 
epileptic  attack  or  consist  of  a  violent  general  shaking 
of  the  whole  body.  A  variety  of  initial  minor  disorders 
usher  in  the  attacks:  the  temperature  is  often  increased. 
The  attacks  are  over  after  one  or  at  most  after  a  few 
hours.  Kraepelin  speaks  of  one  that  lasted  14  days. 
Sometimes  a  status  paralyticus  develops,  suggestive  of 
the  status  epilepticus.  Another  rarer  form  of  charac- 
teristic seizure  is  the  apoplectiform,  which  can  hardly 
be  told  from  an  ordinary  stroke,  and  may  be  followed 
by  the  usual  post-apoplectic  phenomena.  A  good 
many  of  the  strokes  leading  to  sudden  death  in  middle 
life  are  probably  cases  of  neurosyphilis  although  often 
set  down  as  early  arteriosclerosis  of  a  non-syphilitic 
nature.  Besides  the  epileptiform  and  apoplectiform 
seizures,  there  are  certain  seizures  of  a  less  definite  and 
complete  nature,  ranging  from  simple  fainting  spells, 
dizzy  spells  and  petit  mal  attacks,  to  various  special 
forms  of  irritative  muscular  contractions  and  temporary 
speech  disorders.  Sometimes  these  attacks  occur  with 
complete  preservation  of  consciousness.  Transient  par- 
esthesias,  visual  field  defects,  and  especially  attacks  of 
vomiting,  which,  according  to  Kraepelin,  may  precede 
paresis  by  years  (of  course  in  this  connection  gastric 
crises  of  tabes  must  be  thought  of) ,  may  be  counted  as 
sensory  seizures. 

3.  What    is    the    proportion    of    paretic    cases    developing 

seizures?  Figures  vary  from  30  to  90%.  According  to 
Kraepelin,  seizures  occurred  in  30  to  40%  of  his  cases 
at  Heidelberg;  he  was  of  the  impression  that  treatment 
in  bed  had  reduced  the  number  of  seizures.  65%  of 


IIO  SYSTEMATIC  DIAGNOSIS 

paretics  admitted  to  Munich  (under  very  free  conditions 
of  admission)  were  determined  to  have  shown  seizures 
before  their  admission  to  the  hospital.  Seizures  are 
said  to  be  somewhat  more  frequent  in  men  than  in 
women.  These  paretic  seizures  are  not  due  to  either 
hemorrhages  or  vascular  plugging  —  at  least  in  the 
vast  majority  of  cases  —  and  must  be  ascribed  to  the 
effects  of  microscopic  injuries. 

4.  What  is  the  effect  of  seizures  upon  the  future  course  of 

paretic  neurosyphilis?  The  current  idea  as  expressed, 
for  example,  by  Mercier,  is  that  "  immediately  after 
each  crisis  the  patient  is  much  worse  than  he  was  before 
it,  and  thereafter  there  is  some  improvement,  but  he 
never  improves  up  to  the  point  at  which  he  was  before 
the  occurrence  of  the  crisis."  That  is,  "  The  course  of 
the  disease  is  one  of  sudden  plunges,  each  deeper  than 
the  last,  each  followed  by  a  gradual  recovery  that  is  less 
complete  than  the  recovery  from  the  previous  plunge." 

5.  During  what  period  of  the  disease  are  seizures   most 

common?  Late  in  the  disease  many  cases  have  convul- 
sions, even  though  there  were  none  for  the  first  year 
or  two.  In  other  cases  the  convulsion  is  the  first 
indication  of  paresis. 


SYSTEMATIC  DIAGNOSIS  III 


DIFFUSE  (non-paretic)  NEUROSYPHILIS  ("  cer- 
ebrospinal  syphilis  ")  is  often  marked  by  APHASIA. 


Case  21.  Martha  Bartlett,  a  woman  of  40  years,  was 
brought  to  the  Psychopathic  Hospital  aphasic,  or  at  least 
unable  to  talk  distinctly  enough  to  be  understood,  or  even 
to  give  name  and  address.  The  police  had  found  her  wan- 
dering aimlessly  about  the  streets.  Although  she  was  well- 
dressed,  she  was  mud-bespattered  and  apparently  had  not 
changed  her  garments  for  several  days.  It  shortly  developed 
that  the  patient,  although  unable  to  express  herself  either  in 
words  or  by  writing,  could  understand  everything  that  was 
said  to  her  and  could  indicate  by  the  monosyllables  yes  or  no 
whether  she  agreed  or  disagreed  with  statements  made. 
It  was  thus  determined  that  she  was  pretty  well  oriented. 
She  was  able  to  understand  both  speech  and  printed  words. 
Although  she  approximated  more  than  is  at  all  common  a 
pure  type  of  motor  aphasia,  it  appeared  that  there  was  a  slight 
involvement  on  the  sensory  side,  especially  in  the  sphere  of 
visual  imagery. 

Neurologically,  the  patient  showed  moderate  strabismus, 
slight  deviation  of  the  tongue  to  the  right,  and  considerable 
tremor  on  protrusion  of  the  tongue.  The  right  side  of  the 
palate  hung  lower  than  the  left.  The  ankle  and  arm  reflexes 
were  possibly  more  active  on  the  left  side,  and  the  left  grasp 
was  somewhat  better  than  the  right.  Both  knee-jerks  were 
active,  but  again  the  reflex  on  the  left  side  was  more  active 
than  the  right.  No  other  abnormalities  of  reflex  were  deter- 
mined. There  was  no  Rombergism  but  the  gait  was  some- 
what ataxic.  For  the  rest,  the  physical  examination  was 
normal.  The  blood  pressure  was  120  systolic,  85  diastolic. 

The  ready  suspicion  was  that  the  case  was  one  of  apoplexy 
of  slight  degree  with  post-apoplectic  phenomena.  Upon 
investigation,  this  suspicion  was  confirmed  since  it  appeared 
that  Mrs.  B.  had  been  apparently  quite  well  until  about  six 
months  before  admission,  when  without  particular  warning 


112  SYSTEMATIC   DIAGNOSIS 

CONDITIONS  IN  WHICH  SPEECH  DEFECT 
IS  FOUND 

NEUROSYPHILIS 

HYPOGLOSSAL  PARALYSIS 

FACIAL  PALSY 

PARALYSIS  OF  PALATE  (Posr  DIPHTHERITIC) 

BULBAR  PALSY' 

PSEUDOBULBAR  PALSY 

MYOPATHY— FACIO-SCAPULO-HUMERAL  TYPE  OF  LAN- 
DOUZY  AND  DEJERINE 

MYASTHENIA  GRAVIS 

FRIEDREICH'S  ATAXIA 

LARYNGEAL  TABES 

ALCOHOLIC  INTOXICATION 

POST  HEMIPLEGIC 

LENTICULAR  DISEASE 

BILATERAL  ATHETOSIS 

MULTIPLE  SCLEROSIS 

DEAF  MUTISM 

PARALYSIS  AGITANS 

CHOREA 

STAMMERING 

TICS 

HYSTERICAL  APHONIA 

CHART  17 


SYSTEMATIC  DIAGNOSIS  113 

she  began  to  act  strangely  and  promptly  fell  into  a  series  of 
convulsions.  These  convulsions  would  begin  with  twitchings 
of  the  face,  and  then  spread  throughout  the  body.  There 
would  be  a  period  of  unconsciousness  for  two  or  three  hours. 
It  is  not  certain  how  many  of  these  convulsive  seizures  the 
patient  had.  At  all  events  she  is  reported  to  have  recovered 
therefrom  completely,  remaining  well  for  three  months ;  where- 
upon, suddenly,  while  visiting  a  friend,  she  suffered  a  paraly- 
sis of  the  left  side  of  the  body.  She  remained  dazed  and 
had  hospital  treatment  for  about  a  week.  Ever  since  this 
left-sided  paralysis,  the  aphasic  condition  above  described 
has  persisted. 

Such  a  phenomenon  has  often  been  dismissed  in  the  past 
as  due  to  an  early  arteriosclerosis,  but  most  neurologists  and 
internists  of  today  would  look  beyond  the  diagnosis  of  mere 
arteriosclerosis  and  consider  syphilis.  The  only  suggestive 
feature  in  the  case,  aside  from  the  post-apoplectic  reflex 
disorder  and  spastic  phenomena,  is  the  irregularity  and  di- 
minished light  reaction  of  the  pupils.  Our  suspicions  were 
confirmed  by  the  positive  serum  W.  R.  The  W.  R.  of  the 
spinal  fluid  proved,  however,  to  be  negative.  There  was  a 
moderately  strong  gold  sol  reaction  of  the  syphilitic  type. 
There  was  a  slight  excess  of  albumin,  and  there  was  an  ex- 
ceedingly slight  amount  of  globulin.  There  was  but  one  cell 
per  cmm. 

On  the  whole,  it  would  seem  best  to  consider  the  case  of 
Mrs.  Bartlett  to  be  one  of  CEREBRAL  ARTERIOSCLEROSIS  OF 
SYPHILITIC  ORIGIN,  and  a  case  in  which  there  is  no  evidence 
of  meningitis  or  meningo-encephalitis. 

I.  What  is  the  explanation  of  the  negative  spinal  fluid 
W.  R.?  It  may  be  that  none  of  the  W.  R.  producing 
bodies  have  gone  over  into  the  spinal  fluid.  It  has  been 
shown  by  the  work  of  Weston  that  the  W.  R.  produc- 
ing body  is  not  identical  with  the  bodies  responsible  for 
the  other  tests  in  cerebrospinal  syphilis.  Moreover,  it 
has  been  clearly  shown  that  these  several  tests  of  the 
spinal  fluid  do  not  run  at  all  parallel  with  one  another. 
Especially  is  it  true  that  the  chemical  tests  do  not  cor- 
respond at  all  with  the  degree  or  nature  of  the  pleocy- 
tosis.  On  the  whole,  when  involvement  of  the  nervous 


114 


SYSTEMATIC  DIAGNOSIS 


system  is  entirely  vascular,  it  is  not  only  theoretically 
proper  but  also  practically  common,  to  find  a  spinal 
fluid  negative  to  several  tests. 

2.  Omitting  consideration  of  the  syphilitic  gold  sol  of  this 

case,  what  conclusion  could  be  drawn  from  the  albumin 
and  globulin  findings?  It  would  not  be  warrantable  to 
assume  syphilis  since  it  is  a  common  finding  after 
cerebral  hemorrhage  due  to  non-syphilitic  arterioscler- 
osis to  find  excess  albumin  and  also  globulin  in  the 
spinal  fluid.  Occasionally,  also,  pleocytosis  occurs  in 
cases  of  cerebral  hemorrhage  even  when  the  hypothesis 
of  an  active  meningitis  can  be  excluded.  We  may  recall 
in  this  connection  the  pleocytosis  in  so-called  menin- 
gitis sympathica  of  certain  brain  tumors.  (See  also 
the  case  of  Milton  Safsky  (48) ,  a  case  of  brain  tumor  in 
which  there  was  an  excess  of  albumin,  a  large  quantity 
of  globulin,  and  a  pleocytosis  of  146  cells  per  cmm.) 

3.  What  can  be  expected  from  treatment  in  these  cases  of 

vascular  cerebral  syphilis?  The  condition  offers  very 
little  opportunity  for  therapeutic  results.  However, 
antisyphilitic  therapy  is  indicated  to  prevent  if  possi- 
ble further  progress  of  the  lesions.  Since  the  lesions  are, 
however,  vascular,  and  since  it  must  remain  a  question 
how  far  these  vascular  lesions  are  due  directly  to  spiro- 
chetal  action,  and  since  in  any  event  it  may  be  diffi- 
cult to  reach  the  spirochetes  thus  active,  perhaps  it  is 
best  to  place  most  reliance  on  potassium  iodid.  In  any 
event,  potassium  iodid  should  be  given.  Salvarsan 
and  mercury  are  also  indicated.  It  is  common  to  warn 
against  administration  of  large  doses  of  salvarsan  in 
this  type  of  case  on  the  ground  that  further  vascular 
ruptures  may  be  produced.  (See  Friedberg,  108.) 

4.  If  we  conclude  that  the  aphasia  of  the  Bartlett  case  is 

due  to  vascular  disease,  can  we  conclude  a  relation 
between  this  vascular  disease  and  vascular  tension?  It 
is  not  safe  to  draw  such  a  conclusion.  The  Bartlett  case 
itself  showed  low  blood  pressure.  To  be  sure,  some 
cases  of  neurosyphilis  show  high  blood  pressure  from 
which  one  draws  the  &  la  mode  clinical  conclusion  to  the 
effect  that  the  kidneys  are  probably  involved  in  the 
arteriosclerosis;  but  other  cases  do  not  show  a  high 
blood  pressure  but  may  in  fact  show  a  low  blood  pres- 
sure. The  vascular  disease  doubtless  responsible  for 
the  aphasia  in  the  Bartlett  case  is  probably  not  at  all 
an  effect  of  blood  pressure  conditions,  but  is,  on  the 
contrary,  an  effect  of  local  syphilitic  vascular  lesions. 


SYSTEMATIC  DIAGNOSIS  115 


PARETIC  NEUROSYPHILIS  ("  general  paresis  ") 
is  often  marked  by  APHASIA. 


Case  22.  Meyer  Levenson,  a  traveling  salesman  of  36 
years,  had  for  the  last  two  or  three  years  been  undergoing  a 
change  of  disposition,  quite  interfering  with  his  work.  He 
had  begun  to  take  unreasonable  aversions  to  people,  had 
become  irritable  and  emotionally  depressed,  and  often  fell  to 
weeping  without  cause. 

About  nine  months  before  hospital  observation,  it  seems 
that  a  trunk-cover  had  fallen  on  Levenson's  head,  and  there 
is  some  question  as  to  whether  he  did  not  have  a  convulsion 
at  that  time.  However,  a  month  later  he  had  a  definite 
seizure,  followed  by  speech  disorder,  a  slight  paralysis,  and  a 
staggering  gait.  Four  weeks  later,  however,  he  had  gotten 
over  these  post-convulsive  difficulties  and  had  gone  back  to 
work. 

At  his  work,  he  became  tired  easily,  his  gait  and  speech 
did  not  seem  entirely  normal,  and  there  was  a  considerable 
memory  disorder.  After  five  more  months,  another  attack 
of  a  convulsive  nature,  with  twitching  of  hands  and  face  and 
tongue-biting  occurred,  and  the  attending  unconsciousness  re- 
mained for  two  days.  Again  improvement  followed,  though 
without  ability  to  return  to  work.  Four  (?)  months  later 
there  were  several  severe  convulsions  and  Levenson  would 
remain  unconscious  for  a  day  or  two  at  a  time.  Restless- 
ness, irritability,  and  irrational  talking  followed. 

Physically,  the  patient  was  fairly  well  developed  and  nour- 
ished; blood  pressure  1 68  systolic,  68  diastolic;  pupils  re- 
acted very  sluggishly  to  light.  There  was  a  marked  motor 
aphasia,  which  the  patient  recognized  as  a  speech  difficulty. 
On  the  whole,  however,  Levenson  was  very  euphoric  and  was 
entirely  sure  that  he  was  improving  and  would  surely  get  well. 

Shortly  after  entrance,  Levenson  had  a  severe  convulsion, 
with  unconsciousness.  The  movements  were  mainly  on  the 
right  side  of  the  body,  and  there  was  a  post-convulsive  weak- 


U6  SYSTEMATIC  DIAGNOSIS 

ness  of  the  right  side  for  several  days,  followed  by  a  slow 
recovery  of  strength. 

The  course  of  the  disease  —  convulsions  followed  by  im- 
provement —  is  very  characteristic  of  a  paretic  onset.  The 
laboratory  findings  were  in  all  respects  confirmatory.  It  was 
rather  striking  that  a  permanent  motor  aphasia  followed  the 
convulsions  in  this  case,  since  the  seizures  of  paresis  do  not 
in  the  vast  majority  of  cases  leave  permanent  paralyses. 
The  course  of  the  disease  continued  to  show  convulsions, 
which  would  in  each  instance  leave  him  at  a  lower  terrace  of 
capacity  than  had  been  before  shown.  The  patient  died 
four  years  after  the  onset  of  symptoms  of  a  general  asthenia. 
With  the  exception  of  the  permanent  motor  aphasia,  this 
case  might  be  regarded  as  a  fairly  typical  one  of  general 
paresis. 

1.  What  is  the  general  nature  of  speech  disorder  in  paretic 

neurosyphilis?  Speech  disorder  is,  along  with  the 
pupillary  changes,  one  of  the  most  important  clinical 
symptoms  in  paretic  neurosyphilis.  There  are  aphasic 
and  articulatory  disturbances.  The  aphasia  that  ac- 
companies paretic  seizures  is  of  a  transient  nature  as  a 
rule.  A  case  with  such  long-standing  motor  aphasia 
as  shown  by  Levenson  is  not  common.  Paraphasia,  with 
incorrect  naming  of  objects,  may  last  longer.  The  so- 
called  "  sticking "  phenomenon  is  often  observed. 
Word  deafness  is  said  to  be  rarer  but  is  difficult  to  test 
on  account  of  the  patient's  dementia.  Agrammatism 
(incapacity  to  form  correct  sentences)  is  sometimes 
observed.  But  the  most  characteristic  disorder  is 
in  the  syllabic  composition  of  words.  Syllables  are 
left  out  ("  medaltricity "  for  medical  electricity),  or 
fused  ("exity"),  or  doubled  ("  electricicity  ").  Be- 
sides the  central  speech  disorders  of  which  the  above 
are  examples,  there  are  disorders  in  articulation,  which 
at  first  occur  as  a  consequence  of  paretic  seizures  or  in 
states  of  excitement,  but  later  become  permanent.  These 
are  divided  into  paretic  and  ataxic  disturbances. 

2.  What  is  the  structural  basis  of  these  forms  of  aphasia? 

It  is  believed  that  they  are  due  to  microscopic  changes, 
not  to  coarse  destructive  lesions. 


I 

[BROOKLINE, 

MASS.] 


"U/X-  tX.fr  <>    ,         [BROOKLINE,  MASS.] 


Mss.  of  Levenson,  case  22.      Paretic  neurosyphilis.    Tremor,  misspelling. 
Metathesis  of  letters  (Bk,  not  Br)  omission  of  letters  (Book). 

A 


a 


^f   ^-Ji—^ 
/ 


[God  save  the  Commonwealtl 
of  Massachusetts] 


Mss.  of  Safsky,  case  48,  brain  tumor.      Tremor  not  marked. 
of  letters.      Wrong  letters  (h  in  hweth). 


Misspelling,  omissic 


Mss.  of  Halleck,  case  31,  cervical  tabes.       No  brain  disorder. 
on  difficulties.     Crowding  of  phrases  result  of  ataxia. 


Pen-holding  and  bearinj 


V^^^^7  ^^ 


Mss.  of  Collins,  case  61,  paretic  neurosyphilis.     One  misspelling  (-chus^etts)  ;  not  psych< 
pathic?     Characteristic  tremor. 


SYSTEMATIC  DIAGNOSIS  117 


REMISSIONS  of  identical  appearance  occur  in 
PARETIC  and  in  DIFFUSE  (non-paretic  meningo- 
vascular)  NEUROSYPHILIS. 


Case  23.  Thomas  Donovan,  a  merchant  44  years  of  age, 
acquired  syphilis  according  to  his  own  story  at  the  age  of  31, 
and  he  was  at  that  time  treated  at  a  ^well-known  watering- 
place  with  mercurial  injections.  Later  he  continued  treat- 
ment under  his  family  physician,  and  at  34  was  pronounced 
cured.  However,  four  years  later  —  that  is  seven  years 
after  his  initial  infection  and  in  his  38th  year — he  had  his 
blood  examined  and  it  proved  positive.  He  was  accordingly 
treated  by  salvarsan  and  his  W.  R.  became  negative.  The 
story  did  not  end  there,  however,  for  at  43,  mental  symptoms 
appeared  of  the  nature  of  depression  and  a  diagnosis  of  paresis 
was  made.  He  was  released  from  the  institution  against 
advice  at  that  time,  and  without  treatment,  made  a  partial 
recovery. 

A  sudden  outburst  of  violence  brought  Mr.  Donovan  to 
the  Psychopathic  Hospital;  he  was  very  surly,  combative, 
and  difficult  to  manage,  standing  6'  2",  and  weighing  210 
pounds.  He  was  oriented  only  fairly  well  and  his  surliness 
was  streaked  with  humor.  He  facetiously  said  that  the 
Psychopathic  Hospital  was  the  largest  hospital  in  the  country, 
and  that  it  was,  in  fact,  a  horse  hospital;  that  he  had  come 
because  he  liked  the  surroundings,  not  to  make  money;  that 
he  was  the  healthiest  man  in  the  world,  never  having  been 
sick;  that  the  Psychopathic  Hospital  was  a  club,  for  which 
you  have  to  get  somebody  to  propose  your  name.  There 
was  amnesia  and  no  knowledge  of  current  events.  He 
regarded  the  food  as  poisoned,  refused  to  eat,  and  was  very 
irritable  and  untidy. 

Physically,  there  were  few  abnormalities,  but  the  pupils 
failed  to  react  either  to  light  or  accommodation,  and  the 
knee-jerks  and  ankle- jerks  were  absent.  There  was  a  slight 
Rombergism.  There  was  a  marked  speech  defect  to  test 


Il8  SYSTEMATIC  DIAGNOSIS 


ATAXIA  OR  INCOORDINATION 

NEUROSYPHILIS 

LESION  OF  PERIPHERAL  SENSORY  NERVES 

DIVISION  OF  POSTERIOR  ROOTS 


SUBACUTE  COMBINED  DEGENERATION 

VESTIBULAR  ATAXIA 

FRIEDREICH'S  ATAXIA 

FAMILY  PROGRESSIVE  HYPERTROPHIC  NEURITIS 

THROMBOSIS  POSTERIOR  INFERIOR  CEREBELLAR  ARTERY 

MARIE'S  HEREDITARY  CEREBELLAR  ATAXIA 

LESIONS  OF  CEREBELLUM,  TUMORS,  ETC. 

WRITERS'  CRAMP 

PREHEMIPLEGIA 

MULTIPLE  SCLEROSIS 

PSEUDO-SCLEROSIS 

HYSTERIA 


CHART  18 


SYSTEMATIC  DIAGNOSIS 


CONDITIONS  IN  WHICH  VERTIGO  IS  FOUND 

NEUROSYPHILIS 

HEAD  TRAUMA 

CEREBRAL  ANEMIA  AND  HYPEREMIA 

MENOPAUSE 

ARTERIOSCLEROSIS 

RENAL  DISEASE 

CEREBRAL  HEMORRHAGE  AND  THROMBOSIS 

INTRACRANIAL  TUMORS 

MULTIPLE  SCLEROSIS 

EPILEPSY   (AuKA) 

TOXIC  CONDITIONS: 

alcohol,  tobacco,  constipation 

PSYCHONEUROSIS 
OCULAR  DISTURBANCES 
EAR  DISEASE 
MENIERE'S  DISEASE 
MIGRAINE 


CHART  19 


12Q  SYSTEMATIC  DIAGNOSIS 

phrases.  Both  serum  and  spinal  fluid  W.  R.'s  were  positive; 
the  fluid  showed  41  cells  per  cmm.,  there  were  large  amounts 
of  globulin  and  albumin,  and  the  gold  sol  reaction  was  of 
the  "  paretic  "  type. 

Salvarsanized  serum  was  injected  intraventricularly  through 
a  trephine  opening  in  the  right  frontal  region.  Injections 
were  made  through  the  corpus  callosum  into  the  third  ven- 
tricle. There  was  progressive  symptomatic  improvement 
after  each  of  four  injections.  In  fact,  after  the  fourth  injec- 
tion the  patient  was  allowed  to  leave  the  hospital  despite 
the  fact  that  there  was  only  a  slight  improvement  in  the 
spinal  fluid  findings.  The  speech  defect  had  entirely  dis- 
appeared. (Speech  defect,  according  to  many  authorities, 
including  Kraepelin,  is  of  very  grave  diagnostic  significance.) 
His  memory  returned.  Mr.  Donovan  is  now  able  to  handle 
figures  rather  extraordinarily  well.  He  now  has  a  good  in- 
sight into  his  delusions  and  tells  stories  about  them  with 
great  humor. 

I.  What  is  the  definition  of  a  remission  in  general  paresis? 
Remissions  form  a  foil  to  seizures;  just  as  seizures 
mark  a  sudden  advance  in  the  severity  of  the  disease 
or  may  even  lead  to  death;  so  remissions  may  cause 
a  sudden  cessation  of  both  mental  and  nervous  phe- 
nomena in  the  disease.  Whereas  the  seizures  occur 
most  often,  according  to  Kraepelin,  in  the  demented 
types  of  paresis,  the  remissions  occur  in  all  cases  except 
in  the  terminal  phase.  Kraepelin  quotes  Hoppe  as  ob- 
serving pronounced  remissions  of  long  duration  in 
17%  of  male  and  15%  of  female  paretics.  Gaupp 
observed  marked  improvement  in  less  than  10%,  and 
very  marked  improvement  indeed  in  only  i%  of  his 
cases.  Kraepelin  states  that  such  improvements  are 
most  frequent  in  agitated  and  especially  in  expansive 
forms  of  paresis,  and  that  they  are  rarer  and  less  com- 
plete in  the  depressive  and  demented  forms.  Sometimes 
the  improvement  occurs  over  night,  although  the  full 
extent  of  the  remission  becomes  complete  only  gradually, 
perhaps  in  the  course  of  months.  The  sensorium  clears, 
the  disorientation  disappears,  the  delusions  retreat, 
and  the  former  delusions  are  treated  as  dreams  and 
imaginations.  There  is  often  a  good  deal  of  persistent 
uncertainty  as  to  events  during  the  height  of  the  disease. 


SYSTEMATIC  DIAGNOSIS  121 

The  nervous  disorders  are  far  more  obstinate  than  the 
mental.  Still,  both  speech  and  writing  may  often 
greatly  improve. 

Cotton  in  New  Jersey  found,  among  127  cases  of 
paresis  diagnosticated  by  modern  methods  during 
seven  years,  that  remissions  occurred  in  but  five,  or 
about  4%,  lasting  from  a  half  to  three  years. 
Does  a  remission  ever  amount  to  a  cure?  The  classical 
case  quoted  in  this  connection  is  one  observed  by 
Tuczek.  This  case  developed  a  picture  of  paresis  in 
1876,  at  the  age  of  36;  and  a  remission,  or  cessation, 
of  symptoms,  occurred  in  1878 ;  but  in  1883,  at  43  years, 
the  patient  developed  a  tabes  without  any  trace  of 
mental  disorder,  which  tabes  gradually  advanced.  By 
the  middle  of  1898,  when  the  patient  was  58,  certain 
symptoms  of  excitement  and  confusion  occurred,  which 
led  to  death  with  dementia,  22  years  after  the  begin- 
'  ning  of  the  disease.  Nissl  pronounced  the  cortex  to 
be  undoubtedly  the  characteristic  cortex  of  a  paretic. 
This  observation  seems  to  indicate  that  a  clinical 
remission  tantamount  to  a  clinical  recovery  may  occur 
without  the  death  of  the  spirochetes  engaged.  This 
observation  is  to  be  held  in  mind  in  connection  with  all 
therapeutic  work  with  neurosyphilis. 

Nonne  states  that  during  his  clinical  experience  of 
19  years  he  had  followed  10  cases  of  paresis  with  ap- 
parent recovery;  but  of  these  ten  cases,  four  had  to 
be  thrown  out  by  Nonne  because  the  apparent  recov- 
eries turned  out  to  be  only  long  and  almost  complete 
remissions,  finally  issuing  in  characteristic  dementia. 
Of  the  remaining  six  cases,  perhaps  two  should  hardly 
be  counted  as  paretic  and  Nonne  rather  preferred  to 
term  them  cases  of  syphilitic  dementia  in  the  sense  of 
a  non-paretic  cerebral  syphilis.  At  the  end,  therefore, 
of  his  review  of  observations,  Nonne  found  himself 
with  four  cases  of  true  recovery  from  paresis. 

Spielmeyer  holds  that  there  is  no  theoretical  reason 
why  paresis  might  not  be  cured,  since  all  the  different 
changes  that  have  been  described  in  the  disease  can 
be  halted,  and  many  of  them  can  be  repaired.  In 
particular,  he  reminds  us  that  the  acute  infiltrative  proc- 
ess, the  neuroglia  reaction,  and  the  phagocytic  action 
of  the  large  mononuclear  cells  are  distinctly  removable 
processes.  (See  discussion  below  under  Section  V, 
for  apparent  cures  and  remissions  occasionally  secured 
under  treatment.) 


I22  SYSTEMATIC  DIAGNOSIS 


REMISSIONS  of  identical  appearance  occur  in 
PARETIC  ("  general  paresis  ")  and  in  DIFFUSE 
(non-paretic)  NEUROSYPHILIS. 


Case  24.  Michael  O'Donnell,  a  laborer  of  48  years,  came 
home,  one  day,  at  5  30,  complaining  of  severe  headache.  His 
wife  told  him  he  should  lie  down  and,  taking  him  by  the  arm, 
tried  to  help  him  to  the  bed.  At  this  moment,  O'Donnell 
lost  control  of  both  left  arm  and  left  leg,  and  fell,  unable  to 
move  but  with  consciousness  preserved.  The  wife  noted 
that  the  left  side  of  his  face  was  drawn  up  and  that  he  drooled. 
He  was  at  once  carried  to  a  general  hospital,  remaining  there 
for  about  three  weeks,  talking  at  random  in  a  delirious  manner 
and  tied  in  bed.  Two  intraspinous  injections  of  salvarsan 
were  given,  and  O'Donnell  showed  considerable  improvement 
and  went  home. 

However,  upon  his  return  from  the  hospital,  he  became 
very  wilful,  would  not  remain  in  bed,  and  on  one  occasion 
actually  took  the  mattress  from  the  bed,  carried  it  to  another 
room,  and  then  returned  to  his  own  room  and  slept  upon  the 
springs.  He  became  irritable  and  emotional,  insisted  upon 
going  to  the  hospital,  did  not  go  there  but  upon  returning 
home  insisted  that  he  had  been  there.  That  night,  O'Donnell 
left  the  house  only  partly  dressed. 

It  appears  that  O'Donnell  had  been  excessively  alcoholic, 
but  that  before  August  15,  when  he  sustained  the  left-sided 
hemiplegia  above  mentioned,  there  had  been  no  symptoms 
except  that  in  February  he  had  once  been  very  dizzy.  It 
appears  that  there  had  been  another  dizzy  spell,  three  nights 
before  the  paralysis,  accompanied  by  a  fall  and  unconscious- 
ness for  about  15  minutes. 

O'Donnell  was  brought  to  the  Psychopathic  Hospital  some 
six  weeks  after  the  paralysis,  complaining  merely  of  a  slight 
headache  and  desirous  of  treatment.  There  were  no  mental 
symptoms  of  any  sort.  Physically,  O'Donnell  was  in  general 
not  abnormal  (there  was  a  slight  pre-systolic  murmur  and 


SYSTEMATIC  DIAGNOSIS  123 


TRANSIENT  OR  FLEETING  PARALYSES 

NEUROSYPHILIS 

MYASTHENIA  GRAVIS 

MYOTONIA  CONGENITA   (THOMSEN'S  DISEASE) 

PARAMYOTONIA  CONGENITA 

MYOTONIA  ATROPHICA 

INTERMITTENT  CLAUDICATION 

OCCUPATION  NEUROSES 

FAMILY  PERIODIC  PARALYSES 

TETANY 

EPILEPSY  MINOR 

HYSTERIA 

MULTIPLE  SCLEROSIS 

APOPLEXY 

CEREBRAL  THROMBOSIS 


CHAR*  20 


124  SYSTEMATIC  DIAGNOSIS 

a  blood  pressure  of  1 90  mm.  systolic) .  The  pupils  were  slight- 
ly irregular,  the  left  larger  than  the  right;  both  reacted 
sluggishly.  Both  ears  were  moderately  deaf;  the  tendon 
reflexes  of  the  left  arm  and  leg  were  somewhat  more  lively 
than  those  on  the  right.  The  systematic  neurological  ex- 
amination otherwise  revealed  no  abnormalities.  The  urine 
was  negative.  The  serum  W.  R.  was  positive  but  the  spinal 
fluid  reaction  was  negative.  There  were  but  2  cells  per  cmm., 
and  there  was  a  very  slight  trace  of  albumin. 

1.  How  shall  we  account  for  O'Donnell's  transient  paralysis? 

We  might  invoke  brain  tumor,  alcoholic  pseudoparesis, 
or  some  form  of  neurosyphilis.  The  diagnosis  of  brain 
tumor  seems  quite  untenable  in  view  of  the  absence 
of  premonitory  symptoms  and  in  the  absence  of  in- 
tracranial  pressure.  As  for  alcoholic  pseudoparesis  it 
is  true  that  the  patient  was  excessively  alcoholic. 

However,  against  these  two  diagnoses  and  in  favor 
of  the  diagnosis  of  NEUROSYPHILIS,  are  the  positive  serum 
W.  R.  and  the  pupillary  reactions  (although  these  are 
short  of  the  true  Argyll- Robertson  phenomenon). 
Dizziness  with  retention  of  consciousness  and  associated 
with  the  paralyses  mentioned  suggests  rather  a  sub- 
cortical  than  a  cortical  lesion.  We  are  inclined  to 
regard  this  lesion  as  probably  THROMBOTIC,  and  to  place 
it  possibly  in  the  region  of  the  internal  capsule.  We 
are  inclined  to  regard  the  phenomenon  as  purely  vascu- 
lar and  as  not  in  this  case  associated  with  an  encepha- 
litis. We  are,  however,  not  entirely  satisfied  with  the 
diagnosis. 

2.  What  shall  be  said  as  to  treatment?    A  full-blown  left- 

sided  hemiplegia  may  be  produced  even  when  the 
thrombotic  lesion  is  itself  exceedingly  small.  It  is 
common  to  explain  this  on  the  basis  that  there  is  an 
area  of  collateral  edema  about  the  small  necrotic, 
thrombotic,  or  hemorrhagic  area  responsible  for  the 
lesion.  In  short,  numerous  neurones  are  functionally 
rather  than  structurally  affected,  or  at  all  events  capable 
of  early  restitution  of  function. 

3.  What  is  the  prognosis  in  such  cases?      It  appears  that 

now  and  again  patients  run  for  several  years  without 
further  trouble,  both  with  and  without  treatment. 
We  are  inclined,  however,  to  advocate  treatment  rather 
than  absence  of  treatment  for  a  variety  of  reasons. 


SYSTEMATIC   DIAGNOSIS  125 

In  the  first  place,  vascular  lesions  may  at  any  time 
become  associated  with  meningitic  lesions,  and  treat- 
ment by  salvarsan  may  perhaps  be  counted  on  to  head 
off  this  process;  secondly,  the  treatment  with  iodids 
may  possibly  aid  in  the  resolution  of  a  local  thrombotic 
process. 

What  are  the  prodromal  symptoms  of  cerebrospinal  syph- 
ilis? According  to  Nonne,  headache,  dizziness,  sleep- 
lessness, mental  symptoms  of  the  irritability  group,  loss 
of  capacity  as  to  mental  work,  whether  severe  or  not, 
and  loss  of  capacity  for  difficult  thinking;  also  impair- 
ment of  memory.  Nonne  does  not  regard  these  phe- 
nomena as  characteristic  of  syphilitic  vascular  disease, 
and  calls  attention  to  the  fact  that  in  every  organic 
disease  the  same  subjective  symptoms  occur.  The 
triad  —  headache,  dizziness,  and  impairment  of  mem- 
ory —  is  for  example  now  counted  as  a  prodromal  symp- 
tom complex  for  arteriosclerotic  apoplexy  (Cramer). 
Of  course,  apoplectic  attacks  occur  without  such  pre- 
liminary symptoms:  particularly,  according  to  Nonne, 
the  nocturnal  attacks. 

Can  the  fleeting  paralysis  be  of  service  in  differentiating 
the  diffuse  from  the  paretic  form  of  neurosyphilis? 
Probably  not.  In  both  forms  transient  paralyses  occur 
as  well  as  the  permanent  ones.  In  general,  however, 
the  transient  paralyses  are  more  frequent  in  paretic 
neurosyphilis,  whereas  the  permanent  ones  occur  more 
often  in  diffuse  neurosyphilis. 


I26  SYSTEMATIC  DIAGNOSIS 


There  are  cases  of  NEUROSYPHILIS  in  which 
the  laboratory  signs  are  positive  but  in  which  there 
are  no  clinical  signs  or  symptoms  (PARESIS  SINE 
PARESI?). 


Case  25.  Richard  Lawlor*  was  admitted  to  the  Psycho- 
pathic Hospital,  October  29,  1914,  being  sent  there  from  a 
general  hospital  where  he  had  gone  on  account  of  a  self- 
inflicted  wound  of  the  wrist,  apparently  made  in  a  period  of 
depression  with  suicidal  intent.  Routine  notes  follow. 

Family  History.  Paternal  grandparents  both  died  of 
heart  disease.  Maternal  grandfather  died  at  seventy-two  of 
dropsy.  Moderately  alcoholic.  Maternal  grandmother  died 
of  shock  at  fifty-six.  Father  died  at  age  of  forty,  after  an 
illness  of  eight  years,  from  heart  disease.  Father  all  his  life 
was  subject  to  fainting  spells  and  headaches.  The  only  pa- 
ternal cousin  died  at  thirteen  months  of  brain  fever.  Mother, 
aged  forty-seven,  is,  to  say  the  least,  eccentric.  Says  "she 
has  several  times  been  given  up  from  tuberculosis."  Two 
maternal  uncles  died  of  tuberculosis,  one  from  rupture,  one 
from  heart  disease.  One  uncle  who  "  doesn't  know  anything 
after  he  has  a  teaspoonful  of  liquor."  Several  other  uncles 
and  aunts  whose  history  is  not  obtained.  Patient  is  mother's 
only  child.  Mother  was  twice  married.  There  were  sev- 
eral miscarriages  by  both  husbands;  patient  child  by  first 
marriage. 

Past  History.  Patient  born  thirty- two  years  ago,  full 
term,  normal  delivery  and  development.  Measles,  mumps, 
and  chickenpox  in  childhood.  Subject  to  headaches  since 
seven  or  eight  years  old.  Kicked  in  the  face  by  horse  at 
seventeen  or  eighteen,  not  considered  serious.  Hit  by  a 
baseball  three  or  four  years  ago,  leaving  him  hard  of  hearing 

*  Reprinted  from  an  article  by  Southard  &  Solomon: 
"  Latent  neurosyphilis  and  the  question  of  Paresis  sine 
paresi."  Boston  Medical  &  Surgical  Journal,  XXIV,  I. 


SYSTEMATIC  DIAGNOSIS  127 

on  left  side.  Married  ten  years  ago;  no  children  because 
he  says  his  wife  needed  an  operation.  He  denies  venereal 
disease  by  name  and  symptoms.  For  past  ten  years  has 
had  attacks  of  depression  lasting  but  a  short  time,  but  quite 
severe.  Never  caused  him  to  quit  work  as  a  barber  and  he 
felt  better  when  working.  His  married  life  he  says  was 
fairly  happy  except  for  his  wife's  extravagances,  and  on  this 
account  he  left  her  a  little  over  a  year  ago,  and  she  has 
applied  for  a  divorce,  which  he  is  willing  that  she  should 
have,  but  does  not  wish  to  give  her  alimony.  He  admits 
moderate  alcoholism. 

Present  Trouble.  Patient  states  that  since  he  left  his  wife 
a  year  ago  he  has  felt  sorry  a  number  of  times.  He  has 
wished  he  had  her  back.  He  has  felt  lonely.  He  has  had 
six  or  eight  periods  of  depression  in  that  time  similar  to  those 
he  has  had  for  many  years,  lasting  two  or  three  days,  and 
sometimes  a  week.  These  were  always  precipitated  by  some 
cause  for  worry.  In  these  attacks  he  feels  nervous,  sleeps 
poorly,  has  little  or  no  appetite,  sweats  during  his  work  and 
everything  looks  black.  Several  times  in  these  attacks  he 
has  had  suicidal  ideas.  Ten  months  ago  he  considered  taking 
corrosive  sublimate.  For  a  little  over  a  week  before  entrance 
to  hospital  he  had  been  out  of  work  and  had  been  "  sporting." 
The  day  before  entrance  he  had  a  telephone  message  from 
his  lawyer  which  upset  him  somewhat  and  he  walked  the 
floor  all  night.  He  had  just  been  shaving  when  the  idea  of 
suicide  came  to  him.  He  sat  down  a  minute  when  suddenly 
the  thought  "  to  hell  with  the  world  "  came  to  him;  he  took 
the  razor  and  slashed  his  wrist.  He  does  not  remember  draw- 
ing the  razor  across  his  wrist.  As  soon  as  he  saw  the  blood 
he  felt  sorry,  called  his  mother,  and  was  taken  to  an  emerg- 
ency hospital  and  then  sent  to  the  Psychopathic  Hospital. 

Physical  Examination.  Patient  is  a  well-developed  and 
nourished  man  thirty-two  years  of  age.  Head  is  normal  as 
to  size  and  shape;  there  are  no  scars  or  marks  of  injury. 
Hair  and  skin  not  remarkable  in  any  way.  Ears  negative 
to  external  examination.  Teeth  well  kept;  two  missing,  sev- 
eral gold  fillings.  Tongue  very  slightly  coated.  Throat 
negative.  Tonsils  easily  visible  without  evidence  of  inflam- 


I28  SYSTEMATIC  DIAGNOSIS 

mation  or  exudation.  Neck,  no  thyroid  enlargement,  no 
abnormal  pulsations,  no  adenopathy.  Chest,  symmetrical, 
expansion  good,  resonant  throughout.  Breath  sounds  trans- 
mitted normally.  No  rales  or  rubs  heard.  Heart,  no  en- 
largement or  cardiac  dulness.  Sounds  of  good  quality,  no 
murmurs  heard.  Rate  regular.  Pulses  equal,  regular  and 
synchronous,  and  of  good  volume  and  tension.  Systolic 
blood  pressure  130,  diastolic  65.  Abdomen,  flat,  soft  and 
tympanitic  throughout;  no  masses;  no  tenderness.  Liver 
edge  not  felt,  below  costal  margin.  Spleen  not  palpable. 
Extremities  negative,  except  for  incised  wound  on  left  wrist. 

Neuromuscular  Examination.  Pupils  are  large,  round,  reg- 
ular, equal  and  react  readily  to  light  and  accommodation. 
No  nystagmus,  strabismus  or  ptosis.  No  weaknesses  or 
paresis  of  facial  muscles.  The  tongue  projects  medially  and 
shows  no  tremor.  The  triceps  and  biceps  reflexes  are  readily 
elicited,  and  are  quite  active,  as  are  the  knee-jerks  and  ankle- 
jerks.  On  one  occasion  it  was  thought  that  the  tendon 
reflexes  were  slightly  more  active  on  the  left  than  on  the  right. 
This  was  never  confirmed;  always  afterwards  found  equal. 
There  was  no  tremor  of  extended  hands.  Abdominal  re- 
flexes not  elicited.  Cremasteric  present  on  both  sides.  The 
plantar  response  is  flexor.  There  is  no  Babinski,  Gordon 
or  Oppenheim.  No  Romberg.  Coordination  tests  well  per- 
formed. No  speech  defect.  No  sensory  disturbances.  Urine 
examination  negative. 

Wassermann  reaction  in  the  serum:  Positive,  with  choles- 
terinized  antigen ;  negative,  with  syphilitic  fetal  liver  antigen. 

Wassermann  reaction  in  fluid  positive  on  two  occasions. 
Examination  of  spinal  fluid,  November  4:  globulin  ++  + ,  albu- 
min +  +  ,  100  cells  per  cubic  millimeter;  large  lymphocytes, 
8  per  cent;  small  lymphocytes,  90  per  cent;  plasma  cells, 
0.7  per  cent;  endothelial  cells,  1.3  per  cent.  November  n, 
globulin  +  +  +  ,  albumin  +  +  +  ,  cells  18  per  cubic  millimeter. 
November  26,  globulin  ++  ,  albumin  ++  ,  cells  92  per  cubic 
millimeter;  large  lymphocytes,  13.1  per  cent;  small  lympho- 
cytes, 82.1  per  cent;  plasma,  1.2  per  cent;  endothelial, 
3-  6  per  cent. 

Gold  sol,  November  4,  5555432100. 

Gold  sol,  November  26,  3332100000. 


SYSTEMATIC  DIAGNOSIS  I2Q 

Mental  Examination.  On  entrance  to  hospital  patient 
seemed  slightly  depressed  and  a  bit  irritable.  This  condition 
lasted  two  days,  after  which  he  was  agreeable  and  apparently 
entirely  over  his  depression.  Even  during  his  mild  depres- 
sion, however,  he  talked  freely.  There  was  no  evidence  of 
retardation.  He  told  his  story  readily.  Orientation  was 
intact.  Memory  excellent.  Educational  knowledge  well  re- 
tained. There  was  no  evidence  of  any  hallucinations  or 
delusions. 

1.  Was  Richard  Lawlor  insane? 

There  was,  then,  on  the  mental  and  physical  ex- 
amination nothing  to  make  a  definite  suggestion  of  a 
psychosis,  and  the  most  one  could  think  of  was  a 
psychoneurosis  or  a  cyclothymia  of  at  least  ten  years' 
duration.  The  findings  in  the  cerebrospinal  fluid  and 
the  Wassermann  reactions,  however,  give  us  material 
for  thought.  Certainly  one  cannot  call  the  man 
insane;  all  who  saw  him  agreed  on  this  point. 

2.  If   Richard   Lawlor  should   some  day  develop   mental 

symptoms,  what  would  be  the  genesis  of  the  new  psycho- 
sis? Though  writers  such  as  Fildes  and  Mclntosh,  and 
Swift,  have  suggested  an  anaphylactic  or  hyperallergic 
explanation  for  the  development  of  symptoms  after  a 
normal  interval ;  such  a  hypothesis  could  hardly  obtain 
in  the  present  case.  The  hyperallergic  hypothesis  for 
the  development  of  tertiary  neurosyphilis  would  run 
to  the  effect  that  in  the  secondary  stages  there  had  been 
a  definite  disease  of  the  nervous  system,  which,  however, 
absolutely  cleared  up,  leaving  no  inflammatory  vas- 
cular or  parenchymatous  relics  of  its  existence.  Noth- 
ing would  on  this  hypothesis  remain  except  a  hyper- 
sensitisation  of  the  tissues.  In  some  later  period  of  the 
now  clinically  normal  person,  one  or  more  spirochetes 
from  a  lesion  outside  the  nervous  system  are  carried 
into  the  nerve  tissues  and  there  set  up  an  anaphy- 
lactic or  hyperallergic  reaction.  It  is  obviously  diffi- 
cult to  prove  the  correctness  or  incorrectness  of  the 
hyperallergic  theory  without  numerous  examinations 
of  the  spinal  fluid,  in  clinically  normal  persons  after  the 
secondaries  have  passed.  The  present  case,  so  far  from 
demonstrating  a  normal  fluid,  demonstrates  a  highly 
pathological  fluid,  even  though  there  are  absolutely 
no  clinical  symptoms  which  could  be  regarded  as  of 


130 


SYSTEMATIC  DIAGNOSIS 

nervous  origin.  The  burden  of  proof  at  the  present 
time  would  seem  to  lie  with  those  who  claim  hyper- 
allergy  in  neurosyphilis.  We  prefer  on  present  evi- 
dence to  think  that  at  the  conclusion  of  the  secondaries 
a  disease  process  often  remains  in  the  nerve  tissues 
despite  clinical  quiescence. 

What  is  the  prognosis  in  the  case  of  Richard  Lawlor? 
The  prognosis  re  neurosyphilis  is  doubtful.  We  have, 
however,  boldly  termed  the  condition  PARESIS  SINE 
PARES!,  meaning  thereby  to  suggest  that  the  patient 
is  in  considerable  danger  of  the  efflorescence  of  a  true 
diffuse  or  paretic  neurosyphilis.  We  have  no  means  of 
telling,  however,  whether  the  positive  symptoms  would 
be  those  of  a  paretic  or  a  non-paretic  neurosyphilis. 
As  data  accumulate  regarding  these  cases  of  paresis 
sine  paresi,  we  may  be  able  finally  to  come  upon  some 
case  in  which  trauma  shall  bring  out  the  clinical  symp- 
toms of  neurosyphilis.  For  discussion  of  this  matter, 
see  the  case  of  Bessie  Vogel  (52)  in  Part  III  of  this  book. 

Should  Lawlor  have  been  brought  to  a  psychopathic 
hospital?  It  is  a  safe  working  rule  to  have  any  person 
who  attempts  suicide  observed.  A  large  percentage  of 
suicides  occur  in  psychotic  individuals  and  a  suicidal 
attempt  is  not  infrequently  the  first  recognized  abnor- 
mality. Immediate  observation  is  a  necessary  safe- 
guard against  another  more  successful  attempt. 


SYSTEMATIC  DIAGNOSIS  13! 


Demonstrates  SYMPTOMS  and  LESIONS  of 
PARETIC  NEUROSYPHILIS  ("general  par- 
esis ").  Autopsy. 


Case  26.  John  Morrill,  49,  an  operative  in  a  mill  town  in 
Essex  County,  Mass.,  was  described  as  a  "  Saturday  night 
and  Sunday  drinker,"  with  a  history  of  very  serious  long 
sprees  at  the  age  of  43.  It  seems  that  he  had  had  what  was 
called  "  sciatica "  at  35,  and  was  treated  in  hospital  for 
seven  weeks  at  that  time.  The  nature  of  this  sciatica  is  in 
doubt,  but  there  was  a  history  of  syphilitic  infection  at  36 
years  (scar  of  glans). 

Morrill  had  been  married  twice,  and  two  of  the  children 
were  dead;  one  daughter  was  described  as  "  very  nervous," 
but  there  were  four  children  under  ten  years  of  age,  all  re- 
garded as  perfectly  healthy. 

Morrill  had  been  a  mill  operative  of  average  capacity,  was 
industrious,  and  had  supported  his  family  despite  alcoholism. 
The  syphilis  had  been  treated  with  reasonable  thoroughness. 

Aside  from  alcoholism,  there  had  been  no  symptoms  up  to 
two  months  before  admission  to  Danvers  Hospital.  Then 
there  had  been  insomnia,  fatigue,  agitation,  eruption  on  foot, 
loss  of  ten  pounds  in  weight,  hypochondriacal  fears,  appre- 
hensiveness  for  the  future  of  the  children,  incoherent  talk; 
and  just  before  admission,  his  talk  was  described  as  foolish. 
He  had  taken  to  running  away  and  hiding  in  bushes  by  a 
pond  and  in  the  cellars  of  other  people's  houses. 

The  patient  was  of  medium  height  and  weight,  with  thin 
grayish  hair  and  grayish  irides;  musculature  was  slender. 
The  face  was  blank  in  expression,  the  teeth  poorly  preserved 
with  atrophy  of  gums,  the  tongue  coated,  and  the  breath 
foul.  There  was  a  gummy  secretion  of  the  eyelids,  an  area 
of  brownish  branny  eruption  over  both  clavicles,  a  number  of 
depressed  scars  over  the  limbs  and  back,  and  another  area 
of  scaly  eruption  on  the  right  heel  and  the  sole  of  the  foot. 
The  heart  area  was  increased,  and  the  sounds  were  faint  at 


132 


SYSTEMATIC  DIAGNOSIS 


the  base,  with  the  first  sound  accentuated  at  the  apex.  The 
urine  showed  a  trace  of  albumin. 

Neurologically,  the  Romberg  position  was  maintained  with 
a  general  tremor  and  fluttering  of  the  eyelids.  In  compli- 
cated movements,  the  patient  was  slightly  ataxic.  The 
pupils  were  irregular,  the  left  being  much  larger  than  the 
right.  There  were  no  light  reactions  to  be  obtained  in  window 
light.  The  reaction  to  accommodation  was  present,  though 
slight.  Vision  was  poor,  J-inch  capitals  could  not  be  read  by 
left  eye  at  reading  distance.  The  knee-jerks  were  diminished 
equally;  the  Achilles  jerks  were  absent;  the  other  reflexes  were 
normal.  Upon  the  sensory  side,  the  patient  gave  a  history 
of  pains  in  the  legs  at  irregular  intervals  for  several  years. 
These  pains  he  described  as  of  a  darting  character.  There 
was  little  or  no  sensory  disorder,  although  the  outer  surface 
of  the  right  leg  required  a  deeper  pressure  to  elicit  sensation. 
There  were  no  disorders  of  muscle  sense. 

If  Morrill  was  to  be  trusted,  he  had  been  born  in  Ireland, 
and  had  come  to  the  United  States  at  the  age  of  17.  He  mar- 
ried at  1 8 ;  there  had  been  seven  pregnancies  by  the  first  wife, 
with  one  stillborn  child ;  one  child  had  died  at  five  weeks. 
The  four  children  by  the  second  wife  were  healthy.  The 
first  signs  of  neuritis  had  occurred  at  45  and  had  received  the 
diagnosis  neuritis,  although  no  connection  between  the 
neuritis  and  the  syphilis  had  been  noted. 

The  patient  entered  the  hospital  July  26,  1904,  and  was 
discharged,  improved,  January  5,  1905.  He  returned  a  little 
more  than  a  year  later,  January  15,  1906,  and  died  March  21, 
1906.  The  total  duration  of  the  disease  from  the  onset  of 
mental  symptoms  may  therefore  be  stated  as  somewhat  under 
two  years.  When  the  patient  appeared  at  the  hospital  the 
second  time,  he  showed  a  positive  Romberg  sign,  an  unsteady 
gait,  an  ataxia  that  still  was  moderate,  and  somewhat  more 
marked  tremors,  involving  fingers,  tongue,  and  face.  He 
was  now  unable  to  read  |-inch  type  with  the  left  eye.  The 
knee-jerks,  formerly  diminished,  were  both  exaggerated,  the 
left  slightly  more  so.  The  Achilles  reaction,  not  obtained 
formerly,  now  appeared  on  the  right  side.  The  pupils  reacted 
as  before.  The  sensory  loss  had  become  more  marked,  since 


SYSTEMATIC  DIAGNOSIS  133 

sharp  and  dull  points  could  hardly  be  distinguished.  Deep 
pinpricks  were  not  felt  in  the  leg,  and  heat  could  not  be  told 
from  cold. 

The  speech  in  1904  had  been  somewhat  defective  ("  truly 
rural"  rendered  as  "tooly  lualal,"  "sifted  soft  thistles"  as 
"  thoft  thsistles  "),  and  there  had  been  little  further  develop- 
ment of  the  speech  defect.  The  handwriting  had  lost  appre- 
ciably in  legibility  and  had  become  much  more  tremulous. 
During  the  first  period  of  hospital  observation  Morrill  had 
what  might  possibly  have  been  visual  hallucinations,  but  it 
was  impossible  to  tell  whether  his  story  of  seeing  his  wife  and 
children  trying  to  get  in  through  the  window  was  hallucina- 
tory or  a  matter  of  fabrication.  Memory  was  decidedly 
imperfect  and  few  details  of  recent  events  could  be  produced. 
The  association  of  ideas  was  almost  a  so-called  "  flight  " 
of  apprehensive,  fearful  ideas,  loosely  connected,  incoherently 
expressed,  and  dealing  chiefly  with  his  work  and  his  children. 
Judgment  was  imperfect;  the  height  of  the  room  was  esti- 
mated as  24  feet,  but  the  height  and  weight  of  persons  were 
estimated  with  fair  accuracy,  and  also  the  length  of  small 
objects,  whose  lengths  were  doubtless  remembered  rather 
than  estimated.  The  estimate  of  time  elapsing  during  a 
medical  examination  was  accurate,  but  the  estimate  of  longer 
durations  involving  over-night  memories  was  hopelessly 
imperfect.  Emotionally,  there  was  a  dulling  of  sensibility, 
an  appearance  of  suspicion  and  apprehensiveness ;  the  patient 
fancied  himself  to  be  in  a  hopeless  condition  as  a  result  of 
syphilis,  but  at  the  same  time  accompanied  his  statement 
of  his  hopelessness  with  laughter.  A  sample  of  his  hypo- 
chondriacal  ideas:  "  I  am  all  gone;  I  am  good  for  nothing; 
I  am  all  gone  now;  I  can't  drink  now;  can't  write  or  talk  at 
all ;  worse  than  when  you  saw  me  first ;  nothing  in  my  inside ; 
all  wrong  through  me  again;  I  aint  got  no  swallow  now; 
I  can't  die  even;  my  heart  aint  much  good;  I  can't  hear 
it  beat;  I  don't  think  it  flutters;  no  life  in  these  hands; 
they  are  all  cold  and  dead  "  (pointing  to  his  arms  and  moving 
them  about).  During  such  a  portrayal  the  patient  laughed 
in  a  silly  way. 

During  the  second  hospital  stay,  Morrill  was  at  first  restless, 


134 


SYSTEMATIC  DIAGNOSIS 


sleepless,  profane,  imperfectly  oriented  for  time,  possibly 
for  place,  and  also  for  the  attendants.  A  few  weeks  later  he 
became  stuporous  and  confused,  and  his  feebleness  and  phys- 
ical exhaustion  were  finally  ended  by  death,  March  21,  1906. 
Death  was  preceded  by  a  semi-comatose  condition;  a  left 
otitis  media  had  developed. 

At  the  autopsy,  it  appeared  that  death  was  due  to  an  early 
bronchopneumonia  associated  with  acute  splenitis  and  doubt- 
less related  to  the  otitis  media  of  the  left  side.  The  body  at 
large  showed,  aside  from  these  acute  lesions,  a  few  chronic 
lesions,  including  slight  scars  of  the  left  apex,  and  chronic 
adhesive  pleuritis,  chronic  diffuse  nephritis,  and  aortic  and 
coronary  syphilis.  The  aorta  showed  slight  linear  and  nodular 
markings,  with  a  single  small  dark  ulcer  in  the  upper  thoracic 
region,  but  the  aorta  did  not  show  the  characteristic  scarring 
which  syphilitic  aortas  often  show.  The  femoral  marrow 
was  of  a  dark  red  chocolate  color.  The  thyroid  appeared 
to  be  smaller  than  normal.  A  slight  sacral  decubitus  had 
developed. 

The  description  of  the  head  (E.E.S.)  is  given  in  full  on 
account  of  the  encephalitic  lesions  shown.  These  encephalitic 
lesions  may  be  summed  up  as  follows: 

Local  cerebral  atrophy  and  sclerosis  of  the  frontal, 
orbital,  and  central  regions,  especially  of  the  left  oper- 
culum  and  left  supramarginal  gyrus. 

Extension  of  sclerosis  to  hippocampal  gyri  with 
effacement  of  substantia  reticularis  alba. 

Slight  chronic  internal  hydrocephalus. 

Granular  ependymitis  (especially  of  floor  of  4th 
ventricle). 

Compensatory  edema  of  frontal  and  central  pia  mater. 

Cerebellar  sclerosis  (culmen  monticuli,  lobus  culminis, 
lobus  cacuminis). 

Spinal   sclerosis    (grossly   evident   in   the   posterior 
columns  of  the  upper  thoracic  region  and  of  the  lumbar 
enlargement) . 
The  details  are  as  follows: 

Head:  — Bald  on  top.  Hair  gray.  Scalp  normal. 
Calvanum  thin,  deeply  excavated  by  arachnoidal  villi 
to  right  of  vertex.  Diploe  absent.  Dura  closely  ad- 
herent in  bregmatic  region.  Dura  of  usual  thickness. 


SYSTEMATIC  DIAGNOSIS  135 

Sinuses  contain  cruor  clot.  Arachnoidal  villi  slight. 
Pia  mater  hazy  and  over  sulcal  veins  porcelain  white 
over  all  of  vertex  except  occipital  poles  and  over  flanks 
(notably  left).  Thickened  also  around  circle  of  Willis, 
over  culmen  monticuli  and  in  posterior  cerebellar  notch. 
Edema  of  pia  corresponding  to  atrophy  of  frontal  and 
central  regions.  Cerebral  atrophy  most  marked  in 
orbital  surfaces  of  both  frontal  lobes,  in  left  area  of 
Broca,  and  in  left  supramarginal  region.  The  ascend- 
ing branch  and  the  ascending  ramus  of  the  posterior 
limb  of  the  left  Sylvian  fossae  both  readily  admit  the 
thumb  by  reason  of  atrophy  of  adjacent  substance. 
Induration  corresponds  closely  with  atrophy,  but  is 
not  more  marked  about  the  left  Sylvian  fossa.  There 
is  sclerosis  of  both  hippocampal  gyri,  with  loss  of  the 
substantia  reticularis  alba.  The  culmen  monticuli  and 
lobus  culminis  are  firmer  than  the  clival  regions,  and 
the  lobus  cacuminis  is  again  slightly  firmer  than  the 
clival  region.  Cerebellum  a  little  softer  than  usual. 
Pia  strips  with  usual  readiness  from  all  regions.  The 
subpial  region  of  the  frontal  lobes  is  a  trifle  grayer 
than  that  of  the  rest  of  cerebrum.  Ventricles  slightly 
dilated.  Surfaces  evenly  sanded.  Floor  of  fourth 
ventricle  shows  numerous  coarse,  closely  set  granules. 
Brain  wt.  1200  grms.  Cord  shows  a  slight  increase  of 
consistence  over  one  or  two  upper  thoracic  segments 
and  in  lumbar  enlargement  corresponding  with  a  slight 
graying  out  of  posterior  columns.  In  places  there  is 
a  suggestion  of  graying  out  also  in  lateral  columns. 
A  few  calcified  plaques  in  posterior  lumbar  pia. 

Analysis  of  these  details  shows  a  number  of  lesions  that 
characterize  paretic  neurosyphilis  (among  others,  granular 
ependymitis,  frontal  atrophy,  chronic  leptomeningitis) ,  but 
the  lesions  are  more  than  merely  frontal,  extending  as  they 
do  back  as  far  as  the  postcentral  regions  on  both  sides,  and 
even  as  far  as  the  left  supramarginal  gyms.  The  cerebellar 
involvement  although  frequent,  can  hardly  be  said  to  be 
characteristic  in  paretic  neurosyphilis.  The  spinal  involve- 
ment is  characteristic  of  a  case  which  is  probably  to  be  re- 
garded as  one  of  taboparesis;  that  is,  of  paretic  neurosyphilis 
following  a  number  of  years  after  the  establishment  of  tabetic 
neurosyphilis.  The  aorta  is  almost  constantly  affected  by 
sclerosis  in  paretic  neurosyphilis.  The  absence  of  diploe  in 


J36  SYSTEMATIC  DIAGNOSIS 

the  skull  is  not  infrequent  and  the  adherent  dura  mater  is 

often  found. 

Microscopically,    the    tissues    showed    the    characteristic 

lesions  of  PARETIC  NEUROSYPHILIS  ;  nerve  cell  destruction, 

fibrillar  and  cellular  gliosis,  lymphocytic  and  plasma  cell 

deposits  about  the  small  vessels. 

I.  What  are  the  clinical  evidences  of  syphilis  outside  the 
nervous  system?  The  brownish  branny  eruptions  of 
the  skin,  the  depressed  scars  and  the  scaly  eruption 
on  right  heel  and  sole  are  very  suggestive  of  syphilis. 
Such  clinical  evidences  of  syphilis  are  very  important 
in  systematic  examination.  Although  the  laboratory 
tests  are  of  the  utmost  assistance  in  the  diagnosis  of 
syphilis,  the  clinical  signs  should  not  be  neglected,  and 
no  physician  should  rest  satisfied  with  laboratory  signs 
alone.  X-ray  diagnosis  of  bone  conditions  sometimes 
succeeds  when  all  other  methods  have  failed. 


SYSTEMATIC  DIAGNOSIS  137 


GUMMA  of  cerebral  cortex  verified  by  operation; 
death. 


Case  27.  The  presenting  picture  in  the  case  of  David 
Tannenbaum  was  that  of  deep  dementia,  in  which  condition 
the  patient  was  brought  to  the  hospital.  There  was  a  meagre 
history  to  the  effect  that  about  four  months  before  admission, 
he  had  lost  his  job  in  a  hotel  through  lack  of  further  work. 
We  heard  that  at  this  time  he  had  begun  to  suffer  with  ex- 
cruciating pains  in  the  head;  at  first,  worse  at  night,  later, 
worse  by  day.  It  appeared  that  this  pain,  though  it  came 
and  went,  was  chiefly  localized  on  the  left  side  of  the  head. 
For  a  fortnight,  Tannenbaum  had  been  dragging  his  legs, 
until  finally  he  had  become  unable  to  walk  at  all. 

Pari  passu  with  these  developments,  Tannenbaum  had 
become  mentally  confused  and  irritable,  and  his  memory  had 
become  untrustworthy.  For  several  days  before  admission, 
an  appearance  of  marked  dementia  was  presented,  with  slow 
incoherent,  or  at  all  events,  irrelevant  words,  and  a  complete 
disorientation  for  person.  However,  his  vison  had  become 
so  poor  that  it  would  have  been  hard  for  him  to  have  rec- 
ognized any  one. 

It  appeared  that  the  family  history  was  entirely  negative; 
that  the  patient  was  without  education  but  had  been  physi- 
cally very  strong,  and  had  been  fairly  successful  at  first  in  the 
junk  business,  and  later  in  the  clothing  business;  but  latterly 
he  had  been  less  fortunate  in  the  clothing  business,  and 
finally  had  to  resort  to  work  as  a  laborer  around  a  hotel. 

His  wife  had  had  eleven  pregnancies  with  but  one  mis- 
carriage. Nevertheless,  out  of  the  eleven  pregnancies,  there 
were  now  but  four  living  children. 

Physically,  Tannenbaum  was  a  rather  small  man;  he  was 
flabby  and  looked  as  if  he  had  recently  lost  weight.  The  skin 
showed  areas  of  pigmentation  on  the  face  and  sides  of  the 
neck,  and  some  dark  copper-colored  circular  areas,  marble- 
size,  in  the  neck  (syphilitic?).  There  was  a  slight  radial 


138  SYSTEMATIC  DIAGNOSIS 

arteriosclerosis.  The  heart  was  slightly  enlarged  with  distant 
and  indistinct  sounds.  There  was  a  small  pedunculated 
growth  on  the  right  side  of  the  abdomen. 

The  pupils  failed  to  react  to  flashlight  but  they  reacted  to 
sunlight.  They  both  were  slightly  irregular  but  were  equal  in 
size,  and  reacted  in  accommodation.  There  was  apparently 
almost  complete  blindness  and  extreme  deafness.  Arm-jerks 
and  knee-jerks  were  absent;  there  was  an  occasional  slight 
response  of  the  left  ankle- jerk,  but  the  right  ankle- jerk  was 
absent;  the  left  abdominal  reflex  was  very  feeble;  the  right 
absent;  the  cremasteric  reflexes  were  absent,  but  there  were 
no  other  abnormalities  in  the  systematic  examination.  Hand 
grips  weak;  gait  awkward,  with  right  leg  held  somewhat  flac- 
cidly. 

It  was  significant  that  percussion  over  the  left  frontal  and 
parietal  regions  was  able  to  elicit  great  pain.  Either  through 
the  patient's  deafness  or  through  sensory  aphasia,  spoken 
language  was  not  understood.  The  serum  W.  R.  was  positive, 
the  fluid  W.  R.  negative. 

Diagnosis :  The  clinical  symptoms  seem  clearly  to  indicate 
syphilis.  The  local  skull  tenderness  and  impairment  of 
vision  might  well  suggest  intracranial  pressure.  Uniting 
these  suggestions,  we  might  automatically  arrive  at  a  diagnosis 
of  cerebral  gumma.  We  have  learned  to  be  rather  cautious 
of  making  a  diagnosis  of  gumma  of  the  brain  through  its 
mere  rarity. 

Decompression  was  suggested  and  executed.  A  deep 
growth  resembling  a  GUMMA,  in  the  view  of  the  surgeon,  was 
discovered.  No  attempt  could  be  made  to  remove  it.  The 
patient  died  without  recovering  consciousness. 

I.  What  is  the  significance  of  the  negative  fluid  W.  R.  in 
this  case  of  cerebral  gumma?  The  W.  R.  producing 
substances  not  infrequently  fail  to  appear  in  the  spinal 
fluid  from  a  gumma  of  the  brain.  The  serum  W.  R. 
was  positive  in  this  case,  but  even  the  serum  W.  R.  may 
be  negative  in  cases  of  gumma,  both  of  the  brain  and  of 
the  body  at  large.  It  must  be  remembered  that  the 
serum  W.  R.  may  be  negative  in  paretic  neurosyphilis 
(general  paresis);  the  serum  W.  R.  is  even  more  apt 
to  be  negative  in  cases  of  gumma. 


Gummatous  meningitis.     Compression  of  hemisphere.      Tissue 
destruction  of  underlying  cortex. 


SYSTEMATIC  DIAGNOSIS  139 

2.  Is  operative  procedure  to  be  advised  in  cerebral  gumma? 

There  are  cases  in  which  the  acute  and  threatening 
symptoms  of  heightened  intracranial  pressure  require 
operative  treatment  simply  because  the  therapeutist 
cannot  wait  for  the  effect  of  antisyphilitic  treatment. 
Moreover,  antisyphilitic  treatment  of  cerebral  gumma 
is  not  always  as  successful  as  that  of  most  syphilitic 
lesions. 

3.  Could  the  intracranial  pressure  be  caused  by  other  syph- 

ilitic lesions  than  gumma?  A  heavy  meningitis  may 
cause  symptoms  such  as  produced  by  an  intracranial 
tumor.  In  such  a  case  one  will  usually  find  evidences 
of  inflammation  in  the  spinal  fluid.  Cysts  caused  by 
syphilitic  lesions  may  also  produce  identical  symptoms. 

4.  What  is  the  significance  of  cranial  tenderness?    Where 

sensitiveness  to  cranial  percussion  is  not  due  to  a  scalp 
lesion  it  is  very  suggestive  of  a  tumor  underlying  this 
point.  A  gummatous  lesion  of  the  cranium  itself,  may 
occur  without  causing  pain  or  increased  sensitiveness. 


J4Q  SYSTEMATIC  DIAGNOSIS 


CRANIAL     NEUROSYPHILIS     (focal     syphilitic 
.extraocular  palsy)  without  mental  symptoms. 


Case  28.  A  chef,  Paolo  Marini,  28  years  of  age,  reported 
that  on  awaking  one  morning,  everything  appeared  double  to 
him  and  that  his  right  eyelid  had  begun  to  drop.  In  the  fol- 
lowing month  Marini  had  begun  to  feel  weak  and  to  have 
difficulty  in  swallowing,  as  well  as  at  times  difficulty  in  breath- 
ing. The  diplopia  was  found  to  develop  when  Marini  looked 
to  the  right.  Mentally,  the  patient  was  in  all  respects  normal, 
and  no  other  physical  signs  were  found  except  the  diplopia 
and  ptosis  above  mentioned.  The  W.  serum  test  was  positive, 
but  the  tests  of  the  spinal  fluid  were  negative. 

Diagnosis:  "  CEREBRAL  SYPHILIS." 

1.  What  is  the  anatomical  cause  of  this  condition?     It  is 

thought  to  be  due  in  a  number  of  cases  to  a  small  dif- 
fuse gummatous  lesion  at  the  basis  cerebri.  In  the  case 
of  Marini  this  lesion  appears  to  have  been  a  little  more 
extensive  and  to  have  interfered  with  the  tenth  and 
twelfth  nerves  also. 

2.  Why  is  the  spinal  fluid  negative  in  such  a  case  as  that  of 

Marini?  Head  and  Fearnsides  believe  that  intra- 
cerebral  lues  is  characterized  by  a  negative  spinal  fluid, 
under  which  circumstance  one  has  always  to  consider 
the  possibility  of  brain  tumor  or  migraine  in  addition  to 
the  suspicion  of  syphilis. 

3.  What  other  causes  besides  syphilis  should  one  consider 

for  the  sudden  diplopia?  Brain  tumor,  multiple  scle- 
rosis, cerebral  arteriorsclerosis,  tuberculous  meningitis, 
trauma  and  migrainous  ophthalmoplegia,  are  not 
infrequently  at  the  bottom  of  this  condition.  Cases 
also  occur  in  which  the  etiology  remains  obscure,  even 
at  autopsy. 
Under  anti-syphilitic  treatment,  Marini  slowly  improved. 


SYSTEMATIC   DIAGNOSIS  14! 


The  SIX  TESTS  in  TABETIC  NEUROSYPHILIS 
("  tabes  dorsalis  ")  may  run  milder  than  in  paretic 
neurosyphilis  ("  general  paresis  ")  and  character- 
istically run  somewhat  like  those  of  diffuse  (men- 
ingovascular)  neurosyphilis ;  in  particular,  the  fluid 
Wassermann  Reaction  and  the  gold  sol  reaction 
are  apt  to  run  milder.  The  clinical  course  of  tabes 
dorsalis  is  protracted  and  the  prognosis  as  to  life 
is  good. 


Case  29.  Mario  Sanzi,  55  years  of  age,  had  been  having 
what  he  called  rheumatism  since  his  43d  year.  This  rheuma- 
tism affected  only  the  hips  and  legs,  had  at  times  been  very 
severe,  and  for  two  years  past  had  been  almost  constant. 
Before  that  time,  pains  had  come  at  intervals,  lasted  a  vari- 
able period,  and  suddenly  disappeared.  They  were  of 
knife- thrust  character,  and  could  probably  be  called  "  lanci- 
nating." In  a  given  attack,  these  pains  would  come  at 
intervals  of  seconds  or  more.  There  was  also  a  certain 
unsteadiness  in  locomotion  and  inability  to  control  the 
vesical  sphincter. 

Physically,  the  patient  was  entirely  normal  so  far  as  could 
be  made  out  except  neurologically.  Argyll- Robertson  pupils, 
absence  of  knee-jerks,  and  ankle- jerks,  Romberg  sign,  and 
characteristic  gait,  left  no  cause  for  doubting  the  diagnosis 
of  TABES  DORSALIS.  The  blood  and  spinal  fluid  both  proved 
positive  to  the  W.  R.,  though  the  W.  R.  in  the  fluid  gave  a 
negative  reaction  with  o.i  cm.  and  became  positive  with 
0.3  cm.  or  more.  The  globulin  was  somewhat  increased  though 
less  markedly  so  than  in  paresis.  The  gold  sol  reaction  was 
"  syphilitic  "  but  weak.  It  is  to  be  noted  that  the  disease  had 
run  a  12 -years'  course  before  a  doctor  had  been  consulted. 
The  primary  infection  occurred  at  32  years,  namely,  II 
years  before  the  symptoms  began.  At  the  time  of  his  pri- 
mary infection,  Sanzi  had  received  several  years  of  treatment, 
chiefly  in  the  form  of  mercury  by  mouth. 


142 


SYSTEMATIC  DIAGNOSIS 


I.  What  is  the  value  of  mercurial  treatment  of  syphilis 
in  the  prevention  of  tabetic  or  other  forms  of  neuro- 
syphilis?  "  Fournier  strove  for  many  years  to  con- 
vince the  medical  profession  that  a  syphilitic  patient 
should  be  treated  for  at  least  two  years  after  his  in- 
fection, whether  the  syphilis  seemed  latent  or  patent. 
The  method  of  treating  only  the  symptoms  he  charac- 
terized as  the  opportunist  method;  treatment  in  the 
absence  of  definite  symptoms  the  preventive  method,  as 
preventing  the  later  manifestations.  That  prolonged 
treatment  does  prevent  is  shown  by  Fournier' s  figures 
analyzing  2396  cases  presenting  tertiary  signs.^  These 
he  divides  into  three  groups:  Group  I,  comprising  1878 
cases,  or  78  per  cent  of  the  whole  number,  having  no 
treatment  or  inadequate  treatment  —  that  is  mercury 
for  less  than  one  year;  Group  2,  comprising  455 
cases,  or  19  per  cent,  having  moderate  treatment  — 
that  is,  mercury  for  one  to  three  years;  and  Group  3, 
comprising  the  remaining  19  cases  which  represent 
only  3  per  cent  of  the  whole  number,  having  treatment 
for  more  than  three  years."  * 

In  the  light  of  what  we  now  know  concerning  latent 
neurosyphilis,  it  would  seem  well  for  patients  to  be 
followed  from  time  to  time  with  the  W.  R.  on  blood 
and  spinal  fluid  after  the  supposed  completion  of  the 
treatment  of  primary  and  secondary  syphilis.  The 
examination  of  the  spinal  fluid  is  not  superfluous,  as 
our  experience  with  the  so-called  paresis  sine  paresi 
abundantly  shows.  At  the  present  day  it  is  not  good 
practice  to  assure  a  patient  that  he  is  cured  after  two 
years  of  ordinary  mercurial  treatment  without  resort 
to  frequent  spinal  fluid  tests,  even  though  the  serum 
W.  R.  be  negative. 

*  Solomon:  "  How  Shall  Latent  Syphilis  be  Treated?  The 
Prophylaxis  of  Syphilis  of  the  Central  Nervous  System." 
Interstate  Medical  Journal,  XXIII,  8. 


SYSTEMATIC  DIAGNOSIS  143 


TABETIC  NEUROSYPHILIS  ("  tabes  dorsalis  ") 
is  often  quite  ATYPICAL  clinically  and  may  even 
show  no  single  symptom  warranting  the  old  clinical 
name  "  locomotor  ataxia." 


Case  30.  Stephen  Green  is  a  case  of  TABES  DORSALIS  with 
active  knee-jerks  and  without  locomotor  or  muscle-sense 
disorder.  When  observed  at  the  age  of  45,  it  appeared  that 
there  were  but  two  complaints :  lack  of  control  of  the  vesical 
sphincter  and  shooting  pains  in  the  legs.  It  appeared  that 
the  urinary  disorder  dated  back  ten  years,  when  there  had 
been  difficulty  in  passing  the  urine.  Sounds  had  been 
passed  at  the  time;  occasionally  there  had  been  incontinence 
during  after  years,  ascribed  by  Mr.  Green  to  the  passing  of 
the  sound.  However,  the  physician  at  that  time  stated  that 
the  incontinence  was  a  symptom  of  tabes  dorsalis.  The 
incontinence  had  recently  become  worse,  especially  marked 
at  night,  though  also  occurring  in  the  day;  much  worse  during 
excitement,  and  very  much  worse  after  taking  alcoholic 
drinks.  Besides  incontinence,  there  is  also  difficulty  at 
times  in  passing  the  urine,  as  well  as  dysuria. 

As  for  the  pains  in  the  legs,  they  had  been  first  noticed 
some  three  or  four  years  ago  and  considered  to  be  mild 
rheumatic  effects.  Now,  however,  they  have  grown  pro- 
gressively worse  and  have  been  the  effective  cause  of  giving 
up  business.  The  pains  are  sharp,  darting,  pinching,  and 
burning,  and  last,  say,  about  a  second  with  an  interval  of 
about  the  same  length.  The  attack  will  continue  sometimes 
for  many  hours. 

There  is  a  strabismus  of  the  left  eye,  ascribed  by  the 
patient  to  an  accident  with  an  umbrella  (there  had  been 
operation  without  relief).  The  pupils  showed  the  Argyll- 
Robertson  effect  and  were  markedly  irregular.  Despite  the 
divergent  strabismus  with  diplopia,  the  eye  movements  were 
well  performed  although  not  in  parallel  axes.  Ankle- jerks 
could  not  be  obtained  even  on  reinforcement,  but  the  knee- 


144 


SYSTEMATIC  DIAGNOSIS 


jerks  were  lively,  and  the  other  deep  and  skin  reflexes  proved 
normal.  The  blood  and  spinal  fluid  tests  were  characteristic 
of  tabes  dorsalis. 

It  appears  that  the  syphilis  was  acquired  by  this  patient 
15  years  before;  that  is,  5  years  before  neurological  symptoms 
began.  Three  courses  of  treatment  had  been  taken  at  a 
well-known  watering-place,  and  mercury  pills  had  been  taken 
for  two  years  by  mouth.  The  patient  is  married;  has  no 
children ;  there  have  been  no  pregnancies. 

1.  What  causes  may  be  assigned  for  the  absence  of  children 

in  the  family  of  a  tabetic?  There  may  be  lesions  of  the 
genital  apparatus  (orchitis,  or  more  specialized  toxic 
lesions).  But  impotence  such  as  characterized  the 
present  case  must  also  be  taken  into  account. 

2.  What  is  the  therapy  for  tabetic  pains?     Pyramidon  is 

nowadays  much  in  favor;  morphine  may  be  used; 
some  authors  recommend  that  the  patients  be  in- 
structed to  chloroform  or  etherize  themselves  slightly 
for  relief  of  the  pain.  Surgery  of  the  nerve  roots 
may  be  resorted  to  in  extreme  cases.  Intraspinous 
therapy,  suggested  by  various  authors,  seems  to  exert 
beneficial  effect  in  many  cases. 

3.  Is  the  lack  of  control  of  the  vesical  sphincter  an  unusual 

initial  symptom?  On  the  contrary,  the  more  careful 
the  clinical  observation,  according  to  some  observers, 
the  more  likely  is  the  examiner  to  find  that  vesical 
symptoms  were  the  earliest  or  among  the  earliest  com- 
plaints of  the  patient.  Baldwin  Lucke  found  sphincter 
disturbances  to  be  initial  in  8J%  of  his  long  Blockley 
series.  He  found  sphincter  disturbance  to  occur  in 
some  stage  of  the  disease  in  67.6%,  being  exceeded  in 
frequency  only  by  staggering  gait  (87.2%)  and  lan- 
cinating pain  (71.6%).  According  to  Lucke,  the  most 
frequent  initial  symptom  is  lancinating  pain  in  the 
lower  extremity,  which,  it  will  be  noticed,  occurred 
also  in  our  case  of  Stephen  Green  as  an  initial  symptom 
along  with  vesical  disturbance.  Lucke's  figures  show 
that  paresthesia  of  the  lower  extremities  (17.6%)  and 
weakness  of ^  the  extremities  (16.4%)  are  the  next  initial 
symptoms  in  frequency. 

4.  Could  the  early  treatment  in  the  case  of  Stephen  Green 

be  considered  as  adequate?  No  better  answer  can  be 
given  to  this  question  than  by  quoting  from  Dr.  Joseph 


SYSTEMATIC  DIAGNOSIS  145 

Collins,*  who  probably  has  done  more  than  any  other 
one  man  in  this  country  in  insisting  on  the  need  of 
proper  treatment  of  syphilis.  As  to  the  adequate  treat- 
ment of  syphilis  he  says: 

"  It  consists  in  the  proper  use  of  salvarsan  and  mercury 
begun  at  the  earliest  possible  moment  after  infection 
and  kept  up  till  all  biochemical  evidence  of  the  disease 
has  ceased,  while  the  metabolism  of  the  individual  is 
maintained  as  nearly  normal  as  possible.  But  the  phy- 
sician does  not  do  his  whole  duty  when  he  has  accom- 
plished this.  He  must  solicitously  watch  the  individual 
to  see  that  no  evidence  reappears  for  months  and  even 
years  after  the  apparent  cure.  As  an  index  of  such  reap- 
pearance the  Wassermann  test  of  the  blood  serum  and 
of  the  cerebrospinal  fluid  is  the  safest  guide. 

Until  there  is  a  definite  unanimity  of  belief  among  phy- 
sicians as  to  when  the  treatment  of  syphilis  shall  be 
begun,  and  some  concert  of  action  as  to  what  consti- 
tutes the  adequate  treatment  of  syphilis,  we  cannot 
hope  to  make  any  considerable  progress  in  the  preven- 
tion of  syphilis  of  the  nervous  system,  save  by  educating 
the  individual  toward  infection." 

*  Joseph  Collins:  Syphilis  of  the  Brain,  Journal  American 
Medical  Association,  July  10,  1915,  Vol.  LXV,  pp.  139-144. 


SYSTEMATIC   DIAGNOSIS 


TABETIC  NEUROSYPHILIS  may  produce  symp- 
toms chiefly  if  not  entirely  in  the  region  supplied 
by  the  CERVICAL  plexus  ("  cervical  tabes  ")• 


Case  31.  Paul  Halleck,  35,  was  a  salesman  who  had  begun 
to  find  it  hard  to  carry  his  sample  case,  since  he  was  unable 
to  tell  whether  or  not  he  had  it  in  his  hand.  There  was  not 
only  an  anesthesia  of  the  hands,  but  they  felt  numb  and 
there  was  often  a  tingling  sensation.  Of  late  it  had  become 
hard  for  Halleck  to  dress  himself  or  to  write,  and  these  symp- 
toms had  been  slowly  growing  worse.  There  was  no  other 
complaint.  There  was,  however,  a  history  of  a  chancre  about 
7|  years  before,  which  had  been  followed  by  a  rash  and  a 
sore  throat.  There  had  been  treatment  with  mercury  and 
potassium  iodid  alternating  for  a  period  of  two  years. 

Physically,  there  was  no  evidence  of  disease  except  neuro- 
logically.  The  pupils  were  unequal  (the  right  larger  than  the 
left)  and  reacted  slowly  to  accommodation  and  not  at  all 
to  light.  A  marked  ataxia  of  the  hands  was  shown  in  coat- 
buttoning.  The  finger-to-nose  test  showed  a  marked  dys- 
metria.  Arm- jerks  as  well  as  knee-  and  ankle- jerks  were 
absent.  There  was  a  slight  swaying  in  the  Romberg  posi- 
tion but  no  true  Romberg  sign.  There  was  no  difficulty  in 
locomotion.  Both  blood  and  spinal  fluid  proved  positive 
to  the  W.  R.;  globulin  and  albumin  were  increased.  The 
gold  sol  reaction  was  syphilitic,  and  there  were  85  cells  per 
cmm. 

This  case  is  probably  not  a  pure  example  of  CERVICAL 
TABES,  since  the  knee-jerks  are  also  absent,  and  we  may 
suppose  a  degree  of  lumbar  spinal  cord  changes  in  addition  to 
the  cervical  changes.  It  well  illustrates,  however,  that  the 
tabetic  involvement  of  the  cord  may  be  quite  generalized  and 
that  it  may  strike  high  as  well  as  low. 


SYSTEMATIC  DIAGNOSIS  147 


ERB'S    SYPHILITIC    SPASTIC    PARAPLEGIA. 


Case  32.  Margaret  Neal,  a  maid-of-all-work,  36  years 
of  age,  was  committed  to  a  home  for  inebriates  on  account 
of  her  excessive  alcoholism,  but  she  was  shortly  transferred 
to  the  Psychopathic  Hospital  on  account  of  difficulty  with 
locomotion.  We  found  a  very  marked  spasticity  in  walking, 
with  a  characteristic  scissors  gait.  The  pupils  were  some- 
what irregular,  and  although  both  reacted  to  light,  the 
left  reacted  far  more  slowly  than  the  right  and  the  reaction 
failed  to  hold  well.  The  arm  reflexes  were  very  active,  and 
the  knee-jerks  and  the  ankle-jerks  were  particularly  exag- 
gerated. There  was  a  double  Babinski  reaction,  as  well  as 
Oppenheim  and  Gordon  reflexes  and  a  bilateral  ankle-clonus. 
There  seemed  to  be  tenderness  over  the  nerve  trunks  in  the 
back  of  the  leg,  below  the  knee.  There  was  no  evidence  of 
incoordination,  no  Rombergism,  no  disturbance  of  sensation, 
no  disorder  of  the  special  senses,  and  not  even  a  tremor  of 
the  tongue  or  hands. 

Mentally,  the  patient  was  entirely  negative. 

Diagnosis:  Symptomatically,  it  is  entirely  clear  that 
the  patient  was  suffering  from  SPASTIC  PARAPLEGIA.  One 
would  have  to  consider  besides  spinal  syphilis,  also  amyo- 
trophic  lateral  sclerosis,  syringomyelia,  and  spinal  cord 
tumor.  However,  there  appeared  to  be  no  definite  wasting 
of  muscles,  and  the  fact  that  the  sensations  were  intact  seems 
to  rule  out  also  syringomyelia.  There  was  none  of  the 
characteristic  pain  associated  with  a  cord  tumor.  There  was, 
in  fact,  a  strong  clinical  premonition  that  the  case  was  one  of 
spinal  syphilis,  simply  because  syphilis  is  the  most  common 
cause  of  spastic  paraplegia  in  the  adult.  The  pupillary 
anomalies  were  also  highly  suggestive. 

The  serum  W.  R.  proved  to  be  weakly  positive,  as  was  also 
the  gold  sol  reaction  in  the  zones  characteristic  of  syphilis. 
The  spinal  fluid  examination  yielded  14  cells  per  cmm.  There 


148  SYSTEMATIC  DIAGNOSIS 

was  a  positive  globulin  test  and  a  moderate  increase  in  al- 
bumin.    The  W.  R.  of  the  spinal  fluid  was  negative. 

1.  Why  was  the  spinal  fluid  W.  R.  negative  in  this  case  of 

spinal  syphilis?  The  explanation  of  negative  W.  R.'s 
in  spinal  syphilis  is  not  easy.  Possibly,  however,  in 
the  course  of  years  the  intensity  of  the  process  has  been 
reduced  and  possibly  the  W.  R.  has  been  one  of  the 
first  tests  to  disappear. 

2.  How  shall  we  explain  the  nerve  trunk  tenderness?    We 

might  consider  this  to  be  due  possibly  to  an  inflamma- 
tion about  the  posterior  roots.  On  the  whole,  partly 
on  account  of  the  situation  of  the  pains  below  the  knee, 
it  seems  probable  that  the  nerve  trunk  tenderness  of 
this  case  is  the  residuum  of  an  alcoholic  neuritis. 

Treatment :  Under  injections  of  mercury  salicylate,  there 
was  a  rapid  improvement.  In  fact,  in  the  course  of  several 
months,  the  patient  regained  an  ability  to  walk  long  distances. 
There  still  remains  a  certain  spasticity,  but  the  abnormal 
spinal  reflexes  above  mentioned  are  no  longer  present. 


SYSTEMATIC  DIAGNOSIS  149 


SYPHILITIC  MUSCULAR  ATROPHY,  probably 
due  either  to  spinal  parenchymal  lesions,  or  to  root 
neuritis,  or  to  both. 


Case  33.  Joseph  Graham,  now  50  years  of  age,  seemed 
no  longer  to  be  able  to  do  good  work  as  a  teamster.  His 
arms  had  become  weak  and  the  muscles  had  become  tremulous 
and  apparently  wasted.  There  was  also  pain  in  the  left  leg 
and  hip.  It  appears  that  this  latter  symptom  had  been 
thought  to  be  rheumatism,  having  begun  about  8  years  before 
with  a  sudden  sharp  shooting  pain  in  the  left  hip,  about  the 
region  of  the  sciatic  notch.  Graham  had  rubbed  the  hip 
with  liniment,  but  without  reducing  the  so-called  rheumatism. 
The  trembling  of  the  hands  had  begun  some  years  later, 
but  no  wasting  had  been  noticed  except  during  the  past  year. 
The  pain  in  the  leg  had  suddenly  become  so  severe  that  a 
month  before  medical  observation  he  had  quit  work.  The 
question  immediately  arose  whether  Graham  was  not 
suffering  from  some  familial  form  of  muscular  atrophy; 
but  according  to  his  representations,  there  was  nothing  of 
the  sort  in  the  family. 

Physically,  there  was  little  to  note.  Neurologically,  there 
was  more.  The  pupils  were  somewhat  irregular  in  outline, 
and  the  right  was  larger  than  the  left.  The  left  pupil  failed 
to  react  to  light,  and  the  right  pupil  reacted  very  slowly 
and  with  but  a  slight  excursion.  There  was  no  tremor  of  the 
tongue  and  no  evidence  of  facial  palsy  nor  was  there  smooth- 
ing of  the  nasolabial  folds.  It  was  somewhat  remarkable, 
that  in  the  absence  of  these  signs,  there  was  a  marked  speech 
defect.  The  atrophy  of  arms,  forearms,  and  hands  was 
well  marked,  especially  the  atrophy  of  the  thenar  and  hypo- 
thenar  eminences  of  the  right  hand.  The  extended  hands, 
especially  the  right,  showed  a  marked  coarse  tremor.  Fibrilla- 
tion was  found  in  the  muscles  of  the  hands,  forearms,  arms, 
and  pectoral  muscles.  There  was  no  dysmetria,  and  the  dia- 
dochokinesia  was  normal.  Strength  was  diminished  (dyna- 


150  SYSTEMATIC  DIAGNOSIS 

mometer  right  hand,  32  kg.,  left  31  kg.).  There  was  little  or 
no  atrophy  of  the  legs,  although  the  left  thigh  was  perhaps 
slightly  atrophic  and  the  gluteal  muscles  of  the  left  side 
were  somewhat  flabby.  The  patellar  and  Achilles  reflexes 
were  absent  on  both  sides.  There  was  a  slight  swaying  in 
Romberg  position.  Gait  was  normal.  There  was  a  marked 
tenderness  on  the  left  side  of  the  sciatic  notch,  as  well  as 
over  the  entire  distribution  of  both  external  and  internal 
popliteal  nerves.  This  area  of  skin  was  also  hyperesthetic. 
There  were  no  other  neurological  signs  on  systematic  examina- 
tion. 

Diagnosis:  The  sensory  disorder,  the  speech  defect,  and 
the  pupillary  abnormalities  seem  to  render  the  diagnosis 
of  progressive  muscular  atrophy  doubtful.  Nor  was  there 
any  dissociation  of  sensations  to  suggest  a  syringomyelia. 
Under  such  circumstances,  one  must  fall  back  upon  the  ques- 
tion of  syphilis.  Both  blood  and  spinal  fluid  proved  to  be 
positive  to  the  W.  R. ;  the  globulin  was  increased  and  the 
albumin  markedly  so;  there  were  61  cells  per  cmm.,  and  the 
gold  sol  reaction  read  4444321000. 

1.  Is  there  a  relation  of  SYPHILITIC  MUSCULAR  ATROPHY  to 

amyotrophic  lateral  sclerosis?  Spiller,  some  years  since, 
claimed  such  a  relation,  and  it  would  seem  with  some 
justice. 

2.  How   shall    the   present   case   be   classified?    There   is 

evidence  of  root  pains  (left  hip).  We  may  naturally 
suppose  that  these  root  pains  are  reasonably  good 
clinical  evidence  of  a  meningitic  lesion,  of  which  the 
spinal  ^  fluid  clinically  gave  a  confirmation.  The  fi- 
brillation in  this  case  somewhat  suggests,  however,  a 
central  origin  for  the  muscular  atrophy.  Accordingly, 
it  would  be  difficult  to  definitely  classify  the  present  case 
as  either  one  of  meningovascular  syphilis  or  one  of 
central  syphilis.  It  will  be  remembered  that  Head 
and  Fearnsides  classify  muscular  atrophy  under  both 
these  headings. 


SYSTEMATIC   DIAGNOSIS  15! 


The  period  of  SECONDARY  SYPHILIS  is  fre- 
quently (over  a  third  of  all  cases?)  MARKED  BY 
approved  signs  of  NEURO SYPHILIS  precisely  like 
those  of  full-blown  paretic  or  diffuse  (meningo- 
vascular  non-paretic)  neurosyphilis.  These  signs 
occur  sometimes  in  association  with  severe  clinical 
symptoms,  sometimes  without  clinical  symptoms. 


Case  34.  John  Bennett,  28,  was  brought  to  the  Psycho- 
pathic Hospital  much  confused.  His  brother,  who  came 
with  him,  said  that  he  had  been  a  very  heavy  drinker  but  had 
given  up  drinking  about  four  months  before.  He  had 
recently  had  a  cold  but  was  otherwise  in  good  health  up  to 
the  night  before  admission.  On  this  night,  Bennett  had 
become  suddenly  excited  and  went  into  his  mother's  room, 
at  the  common  home,  and  began  to  curse  her.  However,  he 
was  put  to  bed  safely,  but  on  the  next  morning  began  to 
moan  continuously.  After  some  hours  of  moaning,  he  was 
brought  to  the  hospital.  Here  he  remained  difficult  to  manage, 
being  irritable,  noisy,  and  resistive.  Questions  he  either  would 
not  or  could  not  answer,  and  there  was  even  no  evidence 
that  he  understood  questions.  However,  within  a  few  hours, 
it  was  clear  that  he  was  slowly  corning  out  of  the  confused 
state.  On  the  following  day,  it  was  possible  even  to  rouse 
him  and  get  his  name.  The  confusion  gradually  cleared 
still  further  and,  by  the  end  of  three  days,  he  had  become 
mentally  absolutely  well  so  far  as  could  be  determined. 

He  then  informed  us  that  he  had  had  a  chancre  about  five 
or  six  months  before,  followed  by  a  secondary  skin  eruption; 
that  he  had  received  four  injections  of  salvarsan  (the  last, 
a  month  before  admission)  and  three  injections  of  mercury. 
At  about  the  time  of  the  last  injection  of  salvarsan,  he  had 
developed  headache  with  pain  and  slight  stiffness  in  the  back 
of  his  neck;  and  a  fortnight  later,  he  began  to  have  dizzy 
spells,  followed  during  the  last  week  by  difficulty  in  hearing. 
There  was  amnesia  for  everything  that  happened  after  his 


152  SYSTEMATIC   DIAGNOSIS 

spell  of  sudden  excitement  on  the  evening  before  admission, 
and  this  amnesia  was  never  lifted  for  the  four  days  that 
followed. 

Physically,  Bennett  was  very  well  built  and  muscular. 
Nor  were  there  any  evidences  of  disease  outside  the  nervous 
system.  There  was  some  slight  stiffness  of  the  neck  and  slight 
pain  on  movement  of  the  head,  which  probably  ought  to  be 
attributed  to  meningitis.  The  neurological  examination 
showed  tendon  reflexes  all  normal,  and  normal  sensations. 
There  were,  in  fact,  no  neurological  signs  except  that  both 
pupils  were  dilated ;  the  left  was  larger  than  the  right.  Both 
pupils  reacted  to  light  but  reacted  very  poorly.  They  re- 
acted much  better  to  accommodation. 

The  W.  R.  proved  to  be  positive,  as  might  well  be  ex- 
pected in  a  man  whose  infection  had  taken  place  less  than 
six  months  before.  The  globulin  and  albumin  of  the  cere- 
brospinal  fluid  were  in  great  excess,  of  a  degree  which  we 
clinically  express  by  +  +  +  +  .  The  W.  R.  of  the  fluid  also 
was  strongly  positive  down  to  o.i  of  a  cmm.  The  gold  sol 
reaction  was  the  "  paretic  "  type,  and  there  were  228  cells 
per  cmm. 

1.  How  early  may  clinical  evidence  of  neurosyphilis  set  in 

after  infection?  Craig  found  one  case  of  "  brain 
syphilis  "  occurring  one  month  after  infection.  Frye 
claims^  a  case  of  tabes  dorsalis  developing  six  weeks 
after  infection.  Craig  states  that  he  has  had  three 
cases  ^of  brain  syphilis  occurring  within  six  months, 
and  six  within  a  year  of  infection. 

2.  What  effect  ^did  the  salvarsan  injections  have  in  causing 

or  preventing  the  symptoms  in  this  case?  Nonne  sums 
up  the  neurorecidive  question  as  follows:  Since  the 
introduction  of  ^  salvarsan  therapy  for  neurosyphilis, 
paralyses  of  various  cranial  nerves  are  seen  more  fre- 
quently. ^  This  higher  frequency  is  in  part  only  ap- 
parent since  more  attention  has  been  paid  of  late  to 
auditory  and  labyrinthine  disorders.  On  the  whole, 
however  ^  it  must  be  considered  that  salvarsan  does  mo- 
bilize spirochete  foci  which  without  salvarsan  therapy 
would  perhaps  have  remained  latent.  Probably  we  are 
here  dealing  in  some  instances  with  fresh  infections  of 
neurosyphilis,  in  other  cases  with  a  Herxheimer  reaction. 


SYSTEMATIC  DIAGNOSIS  153 

Ehrlich  believed  that  these  latent  foci  occur  particu- 
larly in  places  with  stagnant  blood  current;  as,  for 
instance,  in  the  narrow  bony  canals.  This  hypothesis, 
sufficient  in  some  instances,  is  less  satisfactory  for  cases 
of  peripheral  neuritis,  for  example. 

What  treatment  is  indicated?  Intensive  antisyphilitic 
treatment  is  strongly  indicated.  Whatever  may  be  the 
truth  concerning  the  production  of  neuro-recurrences 
("  neurorecidives ")  it  is  certain  that  the  symptoms 
usually  vanish  with  a  continuance  of  salvarsan  therapy. 
The  important  point  is  to  give  efficient  treatment,  and 
in  a  case  like  Bennett's  improvement  is  fairly  certain 
unless  some  serious  insult  occurs  before  the  remedial 
efforts  have  been  given  time.  It  is  still  an  open  ques- 
tion whether  intraspinous  treatment  is  more  efficient 
in  such  cases  than  intensive  intravenous  injections  of 
salvarsan.  In  Bennett's  case  diarsenol  was  injected 
intravenously  twice  a  week  in  0.6  gm.  doses,  reenforced 
with  intramuscular  injections  of  mercury  salicylate  and 
potassium  iodid  by  mouth.  Under  this  treatment  im- 
provement began  slowly  and  in  a  few  months  he  was 
symptomatically  well  and  after  three  months  his  tests 
were  practically  negative. 


154 


SYSTEMATIC   DIAGNOSIS 


JUVENILE  PARETIC  NEUROSYPHILIS  ("  juve- 
nile paresis  ")  with  OPTIC  ATROPHY. 


Case  35.  Mary  Coughlin,  a  blind  girl  of  16  years,  was 
brought  to  the  hospital  in  a  state  of  great  excitement,  laugh- 
ing and  crying  alternately.  The  neurologist  is  entitled  to 
think  of  blindness,  and  particularly  of  the  optic  atrophy 
which  Mary  showed,  as  probably  due  to  syphilis.  However, 
there  was  no  history  of  syphilis  in  the  father,  who  died  in  an 
accident  at  the  age  of  40,  or  the  mother,  who  died  at  45,  of 
heart  trouble.  An  elder  sister  was  married  and  well;  two 
younger  sisters  were  living  and  well.  The  fifth  sibling,  a 
boy,  had  died  in  infancy.  There  had  been  no  miscarriages. 
In  fact,  the  only  point  in  favor  of  syphilis  was  the  somewhat 
far-fetched  point  that  the  younger  brother  of  the  patient 
had  died  in  infancy. 

The  patient's  history  was  rather  suggestive  of  some  other 
diagnosis.  Her  birth  had  been  normal,  she  walked  and 
talked  at  13  months,  was  at  school  from  six  to  twelve,  reach- 
ing the  seventh  grade,  and  was  considered  bright.  At  three 
years  of  age,  she  had  been  run  down  by  a  car  and  dragged 
under  the  fender  for  a  considerable  distance.  Her  head  was 
hurt  but  the  patient  did  not  lose  consciousness  in  the  accident. 
Fainting  spells  began  at  n,  in  which  spells  the  patient  would 
lose  consciousness  for  a  minute  or  two.  About  this  time,  the 
patient's  eyesight  had  begun  to  fail,  and  for  some  four  years 
she  had  been  entirely  blind.  Headaches  had  come  on  of  late. 

The  Coughlin  case,  except  for  the  above-mentioned  sus- 
picion of  syphilitic  optic  atrophy,  might  be  regarded  as  an 
unusual  example  of  a  post-traumatic  disease. 

We  found  her  to  be  fairly  well  developed  and  nourished; 
there  was  a  deformity  of  the  lower  half  of  the  sternum  and  of 
the  third  and  fourth  ribs  on  the  right  side.  There  were  no 
other  physical  phenomena  found  upon  systematic  examina- 
tion. The  left  pupil  still  reacted  to  light;  the  right  failed  to 
react,  but  this  lack  of  reaction  could  not  be  regarded  as  of 


SYSTEMATIC   DIAGNOSIS  155 

Argyll-Robertson  nature  on  account  of  the  finding  of  optic 
atrophy  with  the  ophthalmoscope. 

Mentally,  it  appeared  that  the  patient's  retention  of 
school  knowledge  was  poor,  though  her  blindness  for  four  years 
had  doubtless  given  her  little  opportunity  to  keep  such  infor- 
mation fresh.  Rather  strangely,  Mary  gave  utterance  to 
many  delusions:  first,  expecting  to  receive  her  sight  by  an 
operation  on  the  head ;  second,  to  write  a  book  of  her  doings ; 
third,  to  buy  a  house  for  the  children;  fourth,  would  pay 
$3000  for  the  house,  earning  the  money  by  working  at  a 
tailor's  or  as  a  trained  nurse;  fifth,  to  go  on  the  stage  to  earn 
money  by  dancing;  sixth,  will  have  lots  of  money. 

One  of  Mary's  characteristic  statements  is  as  follows: 
"  Won't  it  be  lovely  when  I  can  see  Dr.  H.'s  face  in  heaven  or 
some  other  lovely  place?  Dr.  H.  was  a  grand  doctor  to  me, 
and  when  we  get  together  again  we  are  going  to  Tremont 
Temple  and  keep  us  together.  I  am  going  to  do  some  dancing 
and  play  the  piano.  I  am  going  to  graduate  at  the  high 
school  and  go  to  Trinity  College  in  Washington,  and  I  hope 
I  shall  be  a  faithful  keeper  of  mother's  tomb." 

The  patient  was  at  times  euphoric  and  expansive. 

At  this  stage,  what  with  optic  atrophy,  euphoria,  and 
expansive  delusions,  we  should  perhaps  be  entitled,  had  Mary 
been  an  adult,  to  offer  the  diagnosis  GENERAL  PARESIS.  In 
fact,  on  the  whole,  any  other  than  a  syphilitic  cause  for  the 
optic  atrophy  was  exceedingly  doubtful.  Brain  tumor  of  a 
nature  to  produce  optic  atrophy  might  very  improbably  last 
so  long  as  five  years.  There  was  no  evidence  of  any  in- 
toxication at  the  time  when  the  blindness  occurred. 

The  W.  R.  was  positive  in  the  blood  and  spinal  fluid; 
there  was  a  positive  globulin  test,  and  an  excess  albumin 
as  well  as  15  cells  per  cmm. 

1.  What  is  the  significance  of  Mary's  trauma  at  three  years? 

So  far  as  we  are  aware,  none. 

2.  What  light  could  be  thrown  by  a  W.  R.  study  of  the 

family?  In  some  instances,  much  light  is  thrown;  in 
the  present  case  all  three  living  sisters  of  the  patient 
have  been  examined  and  their  serum  W.  R.'s  have  been 
found  negative. 


SYSTEMATIC  DIAGNOSIS 

3.  What  is  the  prognosis  of  juvenile  general  paresis?     Death 

within  a  few  years,  as  in  general  paresis  in  adults. 
The  patients  live  rarely  more  than  four  or  five  years  after 
the  onset  of  symptoms.  Mary  Coughlin  died  a  year 
and  a  half  after  the  above  examination,  namely,  in  her 
eighteenth  year,  some  seven  years  after  the  onset  of 
symptoms. 

4.  What  can  be  said  of  treatment?    A  few  favorable  results 

have  been  reported  after  intraspinous  therapy  (Swift- 
Ellis).  Too  little  work  has  been  done  with  systematic 
treatment  of  juvenile  neurosyphilis,  both  paretic  and 
non-paretic,  to  permit  important  conclusions  at  this 
time. 

5.  How  can  we  explain  the  infection  of  this  sibling  whereas 

the  others,  both  younger  and  older,  escaped?  It  would 
seem  that  we  would  have  to  discard  the  hypothesis  of 
a  congenital  infection  and  consider  that  it  was  acquired 
accidentally  during  the  lifetime  of  the  patient.  Con- 
sidering the  prevalence  of  syphilis  it  is  rather  to  be 
wondered  that  more  such  cases  of  "  innocent  "  infection 
do  not  occur  in  children.  We  may  recall  how  many 
instances  of  juvenile  gonorrhea  occur.  In  a  case  as 
this  where  the  symptoms  calling  attention  to  syphilis 
necessarily  occur  so  long  after  the  original  infection  it 
is  practically  impossible  to  trace  the  origin  of  the 
infection. 


SYSTEMATIC   DIAGNOSIS  157 


The  diagnosis  of  JUVENILE  PARESIS  is  often 
easy. 


Case  36.  Theresa  Mullen,  an  under-sized  girl  of  12  years, 
presented  a  remarkable  appearance  due  to  congenital  am- 
putations of  the  fingers  and  toes.  She  lay  in  bed,  drivelling 
and  making  unintelligible  cries.  It  appeared  that  the  patient 
weighed  about  12  pounds  at  birth  and  was  very  fat;  that 
she  had  been  fed  on  condensed  milk,  had  survived  cholera 
infantum,  whooping  cough,  and,  as  the  parents  said,  "  two 
kinds  of  measles." 

Theresa  had  gone  to  school  at  5  years,  reaching  the  third 
grade  at  the  age  of  9;  but  at  this  time,  she  began  to  lose 
ground  and  was  put  in  a  class  for  backward  children.  More- 
over, at  about  this  time,  the  teachers  noticed  spells  of  cause- 
less laughter  and  meaningless  twisting  back  and  forth. 
Theresa  would  also  scream  at  night,  looking  about  the  room; 
once,  rising  and  crying,  "  Take  him  away,  that  black  thing," 
though  no  appropriate  object  was  present.  There  had  been 
little  or  no  complaint  of  headache.  Theresa  had  been  de- 
teriorating for  some  time,  and  for  a  year  past  had  been 
having  increased  difficulty  in  walking.  For  two  months  the 
child  had  not  spoken  intelligible  words;  for  the  last  week, 
she  had  been  incontinent. 

The  diagnosis  was  almost  obvious  from  the  manual  and 
pedal  deformities  taken  in  connection  with  the  saddle-back 
deformity  of  the  nose.  It  was  interesting  in  connection  with 
the  contentions  of  W.  W.  Graves,  that  the  scapulae  were 
scaphoid  in  type. 

Accordingly,  the  history  given  by  the  parents  seemed 
consistent  enough.  The  parents  were  both  36  years  of  age, 
having  married  at  23.  The  first  pregnancy  was  a  miscarriage 
at  two  months,  of  unknown  cause.  Theresa  came  next; 
thirdly,  came  a  miscarriage  at  three  months;  fourthly,  a 
girl,  who  is  not  strong  or  well  physically,  has  suffered  much 
from  headaches  and  sore  throat,  but  is  fairly  bright.  The 


SYSTEMATIC   DIAGNOSIS 

fifth  pregnancy  resulted  in  a  boy,  who  is  bright  but  of  under- 
size.  Three  more  pregnancies  resulted  in  miscarriage. 

Taking  into  account  the  above-mentioned  physical  charac- 
teristics, the  personal  history,  and  the  family  history  of 
Theresa,  the  diagnosis  could  hardly  be  in  doubt  even  in  the 
absence  of  a  lack  of  pupillary  reaction  to  light  on  the  right 
side,  infantilism  of  genitalia,  positive  W.  R.'s  of  serum  and 
spinal  fluid,  positive  globulin,  and  excess  albumin,  34  cells 
per  cmm.  and  the  paretic  type  of  gold  sol  reaction  which 
were  found. 

The  prognosis  of  this  case  appears  to  be  rapid  deterioration, 
terminating  in  death  within  a  few  months.  Now  and  again, 
however,  some  such  cases  spontaneously  improve.  Such  a 
case  as  that  of  Theresa  Mullen  is  always  disheartening  in 
itself  but  suggests  the  social  value  of  Wassermann  tests  in  the 
other  members  of  the  family.  The  other  children  of  the  Mul- 
len family  proved  to  be  suffering  also  from  syphilis,  since 
their  blood  sera  all  showed  a  positive  W.  R. 

1.  What  is  the  characteristic  age  of  onset  in  JUVENILE 

PARESIS?  An  impression  has  prevailed  in  some  quarters 
that  the  typical  onset  of  juvenile  paresis  is  in  the  ado- 
lescent years,  and  Houston's  first  case  (1877)  developed 
in  a  boy  of  16.  Thierry's  58  cases,  developing  from  the 
8th  to  the  2oth  year,  averaged  14  years  of  age  at  onset. 
Mott's  22  cases  from  the  8th  to  the  23d  year,  averaged 
17  years  at  onset.  According  to  Clouston,  juvenile 
paresis  develops  most  often  at  puberty  (15  to  17  years). 
It  is  sometimes  claimed  that  cases  developing  symptoms 
early  live  longer,  and  that  juvenile  cases  developing 
symptoms  after  the  2Oth  year  run  a  short  course.  For 
a  case  developing  in  the  5th  year,  see  John  Friedreich, 
Case  No.  77. 

2.  What  may  be  concluded  from  the  physical  signs  (con- 

genital amputations)  present  in  this  case  before  the 
development  ^of  mental  symptoms?  Some  cases  of 
juvenile  paresis  appear  to  show  no  physical  signs  what- 
ever in  childhood.  While  these  amputations  might  be 
the  accidental  result  of  a  difficult  delivery,  it  is  more 
probable  that  they  are  due  to  a  syphilitic  process. 


Juvenile  paresis  —  congenital  amputation  of  digits.     This 
case  reached  fourth  grade  in  school  before  deterioration.    > 


SYSTEMATIC   DIAGNOSIS  159 


CONGENITAL  SYPHILIS  is  apparently  capable 
of  producing  simple  FEEBLEMINDEDNESS  (that 
is,  a  form  of  disease  non-paretic,  non-tabetic,  with- 
out special  tendency  to  progression,  and  without 
tendency  to  vascular  insults). 


Case  37.  Isaac  Goldstein  was  a  small  boy  of  six  years  and 
seven  months,  with  a  father  known  to  be  suffering  from  gen- 
eral paresis.  The  child  was  very  irritable  and  nervous  and 
very  difficult  to  manage,  but  would  hardly  have  been  the 
subject  of  medical  attention  except  in  a  family  study  sug- 
gested by  the  paresis  of  the  father. 

The  child  had  been  born  at  term  and  had  apparently 
undergone  a  normal  development.  Physically,  he  showed  no 
definite  signs  of  congenital  syphilis.  In  fact,  the  physical 
examination  was  to  all  intents  and  purposes  negative.  The 
W.  R.  of  the  serum,  however,  proved  to  be  positive.  Mental 
tests  showed  that  his  mental  age  was  that  of  a  child  of  a 
little  over  five  years.  Taking  all  things  into  account,  it  is 
probable  that  he  should  be  regarded,  therefore,  as  somewhat 
retarded  mentally. 

I.  Is  syphilis  answerable  for  the  mental  retardation  in  this 
case?  Provided  that  the  family  is  free  from  feeble- 
mindedness and  mental  disease,  it  would  seem  that 
the  retardation  of  a  congenital  syphilitic  should  per- 
haps be  regarded  as  syphilitic  in  origin.  Of  course,  the 
institutions  for  the  feebleminded  have  not  shown  ex- 
ceedingly high  percentages  of  syphilitic  children  in 
various  W.  R.  surveys;  still,  the  percentage  of  positive 
reactions  in  institutions  for  the  feebleminded  is  clearly 
higher  than  the  incidence  of  congenital  syphilis  shown 
in  the  population  at  large.  Hence,  we  may  conclude 
that  syphilis  is  one  of  the  etiological  factors  in  the 
production  of  feeblemindedness.  Dr.  W.  E.  Fernald, 
of  the  Waverley  School  for  the  Feebleminded,  has  re- 
cently pointed  out  that  the  syphilitic  cases  belong 
rather  in  the  lower  grades  (idiots  and  imbeciles)  of 
feeblemindedness  than  in  the  higher  (morons). 


l6o  SYSTEMATIC  DIAGNOSIS 

2.  Can  we  guess  what  the  pathological  anatomy  and  histol- 

ogy of  the  brain  may  be  in  such  cases?  The  Waverley 
studies  now  in  process  seem  to  indicate  that  some 
cases  have  little  or  no  gross  alterations,  but  show  a  few 
slight  traces  of  lymphocytic  accumulations  discovered 
upon  extended  search,  and  a  certain  tendency  to  the 
appearance  of  rod  cells  in  various  foci.  But  the  whole 
matter  is  still  sub  judice.  It  is  a  question  whether 
these  traces  of  chronic  inflammation  are  the  residuals  of 
a  more  active  process  or  the  beginnings  of  a  process  that 
is  about  to  be  more  active. 

3.  How  characteristic  is  a  positive  W.  R.  in  the  serum  of  a 

child  without  physical  stigmata  of  congenital  syphilis? 
If  we  limit  the  term  stigmata  to  the  major  and  more 
important  signs,  we  must  reply  that  it  is  not  unusual 
to  find  positive  W.  R.'s  in  sera  of  physically  nor- 
mal looking  children.  Except  in  family  studies,  such 
cases  will  often  escape  notice,  either  because  there 
are  no  stigmata  whatever,  or  because  such  stigmata 
as  exist  are  of  a  minor  nature  and  regarded  as  unim- 
portant anomalies.  Some  of  these  cases  occur  in  the 
clinics  later  in  life  as  so-called  syphilis  hereditaria  tarda. 
If  one  wishes  to  discover  these  cases  with  late  de- 
velopment of  symptoms  before  their  full  bloom,  the 
most  obvious  method  is  to  examine  carefully  the  chil- 
dren of  known  syphilitics. 


Scaphoid  Scapulae. 


SYSTEMATIC   DIAGNOSIS  l6l 


JUVENILE  TABETIC  NEUROSYPHILIS  ("  juve- 
nile tabes  ») ;    TREATMENT. 


Case  38.  The  point  in  presenting  Archibald  Sherry,  a 
JUVENILE  TABETIC  of  12  years  on  admission,  is  perhaps 
to  exhibit  pride  in  therapeutic  results. 

There  was  little  or  no  doubt  of  the  diagnosis;  in  an  adult, 
the  phenomenon  would  be  called  tabes  dorsalis  with  a  ques- 
tion of  general  paresis.  The  right  pupil  was  larger  than  the 
left  and  reacted  neither  to  light  nor  to  distance.  There  was 
a  slight  tremor  of  the  tongue  and  of  the  outstretched  hands. 
The  knee-jerks  and  ankle- jerks  could  not  be  obtained,  nor 
could  the  periosteal  reflexes  in  the  legs.  There  was  a  slight 
unsteadiness  in  the  gait  and  in  various  finer  movements,  and 
a  slight  ataxia  of  the  legs.  There  was  not  a  classical  Romberg 
sign  but  there  was  slight  swaying  in  Romberg  position.  The 
teeth  were  Hutchinsonian.  For  the  rest,  the  physical  ex- 
amination was  practically  negative. 

The  family  history  was  of  interest.  On  the  paternal  side 
there  was  nervousness  as  well  as  alcoholism  and  degeneracy. 
The  maternal  grandmother  had  cancer.  Archibald's  father 
was  immoral  and  alcoholic.  There  was  a  girl  four  years 
older  than  Archibald,  who,  though  nervous  and  unstable,  has 
shown  no  signs  or  symptoms  of  syphilis  and  does  not  yield  a 
W.  R.  in  blood  or  spinal  fluid. 

Archibald  himself  was  born  at  term,  a  large  child,  who, 
however,  lost  weight  rapidly,  developing  a  marked  skin 
eruption  on  head  and  back  three  weeks  after  birth.  This 
skin  disease  lasted  for  a  month  and  a  half  and  then  spon- 
taneously disappeared.  Archibald  remained  weak  and  sickly, 
not  walking  until  three  years  of  age.  However,  he  did  well 
in  school  up  to  the  end  of  his  nth  year,  when  he  failed  to 
keep  up  with  the  children.  He  had  been  an  amiable  child 
and  had  gotten  on  well  with  his  playmates.  Some  time  in 
his  loth  year  physical  disability  had  begun;  there  was  numb- 
ness in  the  legs  with  weakness;  at  times,  actual  inability  to 


SYSTEMATIC  DIAGNOSIS 

walk.  The  right  pupil  was  noticed  by  the  mother  to  have 
increased  in  size;  the  eyelashes  had  turned  white.  There 
was  pain  over  the  left  eye  and  a  feeling  of  weight  on  top  of 
the  head.  Speech  became  difficult  or  even  confused. 

Consistently  enough,  the  W.  R.  both  in  blood  and  spinal 
fluid  was  positive.  Globulin  and  albumin  were  present  in 
large  amounts;  there  were  150  cells  per  cmm. 

Granting  that  this  be  in  some  sense  a  case  of  juvenile 
tabes  we  may  raise  a  doubt  whether  the  case  is  one  of  congeni- 
tal syphilis.  The  W.  R.'s  of  the  blood  of  both  father  and 
mother  are  negative.  Syphilis  is  denied  by  them.  The 
nervous  and  unstable  older  sister  failed  to  show  definite 
symptoms  of  syphilis  or  a  positive  W.  R.  There  had  been  no 
miscarriages  or  stillbirths.  The  question  arises  whether  the 
Hutchinsonian  teeth  do  not  indicate  congenital  syphilis.  It 
appears,  however,  that  it  is  possible  to  develop  Hutchinsonian 
teeth  if  syphilis  is  acquired  before  the  teeth  are  formed.  We 
have  no  data  as  to  how  or  why  this  particular  baby  should 
have  acquired  syphilis,  if  he  did  so  acquire  it,  at  the  age  of 
three  weeks.  On  the  whole,  sceptics  may  doubt  our  sug- 
gestion that  the  case  is  one  of  acquired  juvenile  tabes.  Pos- 
sibly the  question  is  academic  so  far  as  treatment  is  concerned. 

Prognosis:  The  rarity  of  juvenile  tabes  is  such  that  little 
can  be  said  as  to  prognosis.  Three  and  a  half  years  have 
passed  since  a  few  injections  of  salvarsan  were  made.  The 
pains  above  mentioned  rapidly  disappeared,  the  gait  became 
steadier,  the  attacks  of  confusion  ceased,  and  the  speech  im- 
proved. Unfortunately,  on  account  of  a  lack  of  cooperation 
on  the  part  of  Archibald's  mother,  we  have  been  unable  to 
continue  treatment.  However,  we  have  from  time  to  time 
followed  the  patient  in  his  home  and  he  seems  to  have  shown 
no  falling  back  after  the  initial  improvement.  It  would  be  of 
great  value  could  we  know  the  situation  in  the  spinal  fluid  at 
the  present  time. 

I.  Is  there  any  explanation  why  paresis  should  occur  in 
some  juveniles  and  tabes  in  others?  There  is  no 
available  explanation  for  this  difference  nor  any  for 
the  characteristic  early  optic  atrophy  of  juvenile 
tabetics. 


Be  frustrate,  all  ye  stratagems  of  Hell, 
And,  devilish  machinations,  come  to  nought! 


Paradise  Regained,  lines  180-181 


III.   PUZZLES  AND   ERRORS   IN  THE 
DIAGNOSIS  OF  NEUROSYPHILIS 

This  part  of  the  case  collection,  dealing  with  puzzles  and 
errors,  is  ushered  in  by  six  cases  (39-44)  drawn  from  a  group 
of  errors  in  diagnosis  made  some  years  since  at  the  Danvers 
Hospital.  These  six  are  autopsied  cases.  Attention  is  called 
to  the  fact  that  modern  methods  of  diagnosis  might  have 
prevented  the  errors. 


DIFFUSE  NEUROSYPHILIS  ("  cerebrospinal 
syphilis")  versus  PARETIC  NEUROSYPHILIS 
("  general  paresis  ").  Autopsy. 


Case  39.  Caroline  Davis,  dead  at  49  years,  was  a  case 
of  error  in  the  diagnosis  of  general  paresis.  Like  Cases 
40  to  44,  Case  39  was  diagnosticated  by  the  full  Danvers 
staff  as  a  case  of  general  paresis ;  however,  it  must  be  added, 
before  the  days  of  the  W.  R.  and  the  modern  methods  of 
systematic  diagnosis.  As  will  transpire  in  the  sequel,  there 
is  a  large  question  whether  Case  39  is  not  after  all  really  a 
case  of  neurosyphilis,  possibly  not  of  the  paretic  group. 
The  details  are  as  follows: 

Caroline  Davis  was  a  normal  school  girl  till  15,  apt  in 
studies,  mill  worker  till  marriage  at  18;  one  child,  dead 
(cause  unknown).  Habits  good.  Moderate  deafness  set  in 
in  the  forties  and  in  1901  patient  became  completely  deaf 
in  three  months'  time.  In  1905  she  became  unable  to  take 
care  of  her  house  and  had  a  shock  in  which  the  right  leg  was 
affected. 

On  commitment  patient  showed  good  development  and 
nutrition  with  slight  enlargement  of  capillaries  of  cheeks, 
redness  and  roughening  of  skin  of  right  ankle.  Teeth 

165 


1 66  PUZZLES   AND    ERRORS 

absent.  Slight  radial  and  brachial  arteriosclerosis.  Urine 
negative.  Sluggish  pupil  reactions  to  light  both  directly  and 
consensually.  Deafness  absolute,  bone  conduction  defective. 
Arm  reflexes  brisk,  knee-jerks  equal,  brisk.  Bilateral  Ba- 
binski  reaction  more  marked  on  the  right  side,  tremor  of 
tongue,  Romberg's  sign,  gait  defective.  Speech  stumbling, 
writing  clear,  without  tremor. 

Communicated  by  writing  only.  Consciousness  normal, 
disorientation  for  day  of  month,  for  place  (misnames  hospital) 
and  for  persons  (recognizing  nurses,  not  patients). 

Patient  wrote  many  letters  complaining  of  pain,  headaches 
and  especially  of  pain  in  the  abdomen  and  side.  The  patient 
was  thought  to  show  a  slight  defect  of  memory,  but  her 
deafness  rendered  diagnosis  difficult.  The  patient  died 
suddenly  on  May  23,  1908,  shortly  after  supper,  falling  back- 
wards, and  dying  in  five  minutes  with  marked  respiratory 
distress. 

Post  Mortem  Findings.  The  cause  of  death  was  not  clear. 
The  heart's  blood  and  cerebrospinal  fluid  were  sterile.  There 
was  a  small  hemorrhage  in  the  anterior  part  of  the  right 
ventricle  derived  from  a  small  artery  of  the  caudate  nucleus. 
There  was  about  400  cc.  of  blood  between  the  dura  mater  and 
the  pia  mater.  There  was  a  slight  sclerosis  of  the  basal  and 
Sylvian  arteries.  The  brain  substance  was  uniformly  softer 
than  normal. 

It  is  possible  that  the  hemorrhage  had  taken  place  some 
time  before  the  patient's  fall  and  that  the  brain  substance 
had  swollen  in  consequence.  Just  before  the  fall  she  had  a 
weeping  spell. 

The  anatomical  diagnoses  were  as  follows : 

Obesity,  unequal  pupils,  fresh  wound  near  left  ear,  edema 
of  legs,  slight  focal  adhesive  pleuritis,  hypostatic  congestion 
of  lungs,  chronic  endocarditis,  chronic  myocarditis,  congestion 
of  kidneys,  congestion  of  pancreas,  subacute  splenitis,  chronic 
adhesive  pelvic  peritonitis,  hematoma  and  cystic  condition  of 
Fallopian  tubes,  calvarium  dense  and  thick,  subdural  hem- 
orrhage, slight  chronic  leptomeningitis,  general  cerebral  at- 
rophy, marked  in  tips  of  frontal  lobes,  old  cyst  of  softening 
between  left  corpora  albicantia  and  optic  chiasm,  small 


PUZZLES   AND   ERRORS  1 67 

punctures  of  left  ear  drum,  drums  opaque,  chronic  spinal 
leptomeningitis ;  brain  weight,  1190  grams. 

There  were  marked  firm  interadhesions  between  dura  and 
pia  throughout.  A  lumbar  puncture  soon  after  admission 
in  1907  had  shown: 

Per  cent 

Endothelial  cells 10 

Lymphocytes 30 

Plasma  cells o 

Phagocytes o 

Polymorphonuclear  cells 51 

Unclassified 9 

Fibroblasts o 

Cells  in  100  fields 125 

It  will  be  noted  that  the  lumbar  puncture  yielded  no 
plasma  cells  and  yet  showed  30%  of  lymphocytes.  Alz- 
heimer, in  1904,  attempted  to  distinguish  the  histology  of  the 
cerebral  syphilitic  from  that  of  the  general  paretic,  main- 
taining that  lymphocytosis  was  the  characteristic  feature  of  the 
ordinary  neuro syphilitic,  whereas  plasma,  cells  were  associated 
with  the  lymphocytes  in  the  paretic.  This  case  showed  lym- 
phocytic  deposits.  To  be  sure,  they  were  decidedly  sub- 
ordinate in  the  cerebral  cortex,  cerebellum,  and  basal  ganglia, 
to  the  marked  evidences  of  nerve  cell  destruction,  although 
there  were  perivascular  infiltrations  about  a  few  of  the  larger 
vessels  in  the  white  matter  of  the  cerebral  cortex. 

The  spinal  cord,  however,  showed  a  most  severe  infiltra- 
tion, especially  in  the  gray  matter,  where  the  infiltration 
accompanied  severe  nerve  cell  changes  and  arterial  changes. 
The  pia  mater  of  the  spinal  cord  was  also  packed  with  mono- 
nuclear  elements,  among  which,  however,  no  plasma  cells 
could  be  found. 

But  although  the  inflammatory  changes  in  the  shape  of 
lymphocytosis  were  relatively  more  prominent  in  the  spinal 
cord  than  in  the  cortex,  yet  the  cortex  yielded  evidence  of  an 
exceedingly  marked  destructive  process.  Perhaps  no  layer 
of  any  of  the  areas  of  the  cortex  examined  failed  to  show  some 
atrophic  alteration.  The  upper  layers  of  the  cortex  were 
everywhere  more  severely  diseased  than  the  lower  layers. 
Here  we  are  dealing  with  an  instance  of  an  active  meningomye- 


1 68  PUZZLES  AND  ERRORS 

litis  and  subcortical  encephalitis.  It  is,  of  course,  probable 
that  the  W.  R.,  had  it  been  performed,  would  have  been 
positive  in  this  case.  On  the  basis  of  the  histology,  we  are 
inclined  to  regard  the  clinical  picture  in  this  case  as  belonging 
among  cases  of  NON-PARETIC  DIFFUSE  NEUROSYPHILIS. 

This  case,  as  also  the  next  several,  is  especially  instructive 
in  teaching  the  difficulty  in  differentiating  paretic  and  non- 
paretic  neurosyphilis.  Not  only  is  this  difficulty  met  in 
clinical  diagnosis,  but  in  pathological  diagnosis  as  well. 

The  histological  diagnosis  depends  in  large  part  on  the 
work  of  the  Nissl- Alzheimer  school,  which  has  received  great 
recognition.  At  the  present  time,  however,  there  is  begin- 
ning to  be  considerable  doubt  as  to  the  entire  validity  of  this 
teaching.  At  any  rate  there  are  many  borderline  cases  in 
which  the  differentiation  is  well  nigh  impossible.  In  this 
case  note  chronic  meningoencephalitis,  with  cortical  degener- 
ation, in  the  absence  of  plasmocytosis. 

From  the  clinical  standpoint  the  intensity  of  the  W.  R., 
the  character  of  the  gold  sol  reaction,  and  the  result  of 
therapy  have  added  new  points  in  differentiation.  Much 
more  work  controlled  by  autopsies  is  still  needed,  however, 
to  put  us  on  sure  ground  in  borderline  cases. 


PUZZLES  AND  ERRORS  169 


VASCULAR  NEUROSYPfflLIS(?)versus  PARETIC 
NEUROSYPHILIS  ("  general  paresis  ").    Autopsy, 


Case  40.  Case  40  like  Case  41  was  an  error  in  the  diag- 
nosis of  general  paresis  which  might  be  regarded  as  academic 
rather  than  practical.  Both  were  cases  of  arteriosclerotic 
brain  disease  with  severe  cerebellar  involvement.  Case  40 
had  a  spinal  cord  that  was  not  quite  normal.  There  was  a 
tabetiform  lesion  in  the  cervical  spinal  cord  (not  elsewhere), 
together  with  a  unilateral  degeneration  suggesting  in  some 
respects  a  radicular  origin.  The  most  striking  feature,  how- 
ever, of  Case  40  as  in  Case  41,  was  a  lesion  of  the  cerebellum. 
In  Case  40  the  dentate  nuclei  were  in  large  part  destroyed 
by  cysts  of  softening,  although  the  cerebellar  cortex  was 
fairly  well  preserved  on  both  sides.  The  details  of  Case 
40  are  as  follows: 

H.  F.,  male,  gear  maker,  born  1850. 

Heredity.  Maternal  grandmother  insane.  Mother  insane 
at  52,  became  demented  and  lost  use  of  limbs,  died  at  71. 
Aunt  insane. 

Personal  History.  Common  school  education.  Capable 
workman  till  within  a  few  months.  Early  in  life  alcoholic. 
Drunk  almost  every  week  until  1899  or  1900.  Irritable, 
nervous,  selfish,  loose  in  relations  with  women.  Venereal 
disease  denied  by  wife.  Married  in  1883.  Three  frail 
children.  No  miscarriages.  Neuralgia  in  1901  or  1902. 

January,  1904,  patient  left  carriage  shop  on  account  of 
mistakes  in  work,  became  more  pleasant,  childish,  fearful, 
talkative,  did  funny  things,  later  became  vagrant,  stole  from 
fruit  stores,  smoked  cigarettes  picked  up  in  the  street,  and 
became  restless  and  irritable. 

Committed  to  Danvers,  June  24,  1904,  with  slightly 
enlarged  heart,  somewhat  heightened  blood  pressure,  and  a 
slight  sediment  of  epithelial  cells  in  urine. 

Romberg's  sign  was  present,  but  there  was  little  or  no 
demonstrable  incoordination  otherwise.  Very  slight  tremor 


170 


PUZZLES   AND    ERRORS 


of  fingers.  Left  knee-jerk  absent,  right  obtained  on  re- 
enforcement.  Achilles  jerk  absent.  Triceps,  wrist  and 
normal  plantar  reflexes  present.  Pupils  react  to  accommoda- 
tion, but  very  slightly,  if  at  all,  to  light.  Sensations  normal 
except  in  legs.  The  legs  show  preservation  of  tactile  and 
temperature  senses,  but  abolition  of  pain  sense  except  over 
dorsum  of  foot. 

Speech  showed  slurring  of  syllables  and  "  brigrade  "  for 
"  brigade."  Disorientation  for  time,  place  and  in  part  for 
persons.  Admitted  that  his  work  had  been  deficient  but 
regarded  himself  as  well.  Emotionally  variable,  crying  at 
times  and  suddenly  becoming  jocular.  Eloped  July  3  and 
somehow  reached  his  wife's  house  in  a  neighboring  city. 

Euphoria  persisted.  The  pupils  continued  Argyll-Robert- 
son, and  the  knee-jerks  remained  absent.  Became  oriented 
for  place  and  partially  as  to  time  (month  and  day  of  week 
correct) . 

During  1905  failure  became  rapid,  with  ataxia  of  legs, 
persistent  euphoria,  and  loss  of  weight. 

Convulsions,  regarded  as  general  paretic,  developed  in 
1906.  Death  sudden,  December  7,  1906. 

Post  Mortem  Findings.  The  cause  of  death  was  strep- 
tococcus septicemia,  probably  derived  from  a  gangrenous 
bronchopneumonia  or  related  with  a  small  thrombus  of  the 
right  auricular  appendix.  There  was  also  an  acute  purulent 
otitis  media,  mastoiditis  and  sphenoidal  sinusitis,  as  well  as 
extensive  decubitus.  From  this  decubitus  or  from  the 
intestinal  tract  may  have  been  derived  the  numerous  colonies 
of  bacillus  coli  communis  which  developed  on  plates  from  the 
cerebrospinal  fluid. 

Arteriosclerosis  was  little  in  evidence,  being  confined  to 
the  coronary,  right  vertebral  and  carotid  arteries  (slight  in 
all).  Cysts  of  softening  existed  in  the  posterior  part  of  each 
dentate  nucleus  and  may  probably  be  interpreted  as  indicating 
vascular  disease. 

Chronic  disease  outside  the  nervous  system  was  prominent 
and  in  part  suggestive  of  senile  findings;  milky  patches  of 
pericardium,  adhesions  about  liver  and  gall-bladder,  adhesions 
about  spleen,  adhesions  and  fibrous  thickening  of  parietal 


PUZZLES   AND   ERRORS  171 

peritoneum,  adhesions  in  both  pleural  cavities,  chronic  diffuse 
nephritis,  hypertrophy  of  bladder  wall,  dense  calvarium,  dural 
adhesions. 

The  nervous  system  showed  several  unexpected  features. 
The  absence  of  chronic  leptomeningitis  was  striking:  the  pia 
mater  was  everywhere  delicate  and  transparent  except  that 
the  walls  of  the  cerebellar  and  chiasmal  cisternae  were  thick- 
ened and  that  there  were  slight  opacities  along  the  sulcal 
veins  of  the  convexity.  Brain  weight  1090  grams.  There 
was  a  generalized  sclerosis  and  pigmentation  of  the  cerebral 
cortex.  The  sclerosis  varied  in  degree  and  was  most  marked 
in  the  prefrontal  regions,  the  anterior  halves  of  the  superior 
frontal  gyri,  the  middle  third  of  the  right  precentral  gyrus, 
the  region  of  the  splenium  on  the  left  side,  and  the  sagittal 
rami.  If  the  bacillus  coli  communis  found  in  the  cerebro- 
spinal  fluid  had  any  effect  upon  the  consistence  of  the  brain, 
obviously  hard  to  prove  in  a  brain  of  leathery  consistence  at 
the  outset,  it  was  shown  only  in  the  right  Rolandic  area  in  the 
vicinity  of  the  sclerotic  part  of  the  precentral  gyrus.  Granu- 
lar ependymitis  of  all  ventricles.  Weight  of  cerebellum,  pons 
and  bulb,  135  grams. 

Perhaps  the  most  remarkable  feature  of  all  in  the  case  was 
the  occurrence  of  cysts  of  softening  in  the  posterior  part  of 
each  dentate  nucleus.  For  discussion,  see  Case  41. 


172  PUZZLES  AND   ERRORS 


VASCULAR  NEUROSYPHILIS  (?)  versus 
PARETIC  NEUROSYPHILIS  ("  general  paresis  ")• 
Autopsy. 


Case  41,  like  Case  40,  was  one  of  arteriosclerotic  brain 
disease  with  severe  cerebellar  involvement.  Here  is  another 
case  in  which  the  Danvers  staff  made  a  diagnosis  of  general 
paresis  without  dissenting  voice.  There  were  some  tabetic 
symptoms,  and  the  spinal  cord  at  autopsy  did  show  a  moderate 
lymphocytic  infiltration  of  the  meninges,  entirely  consistent 
with  the  picture  in  the  spinal  fluid.  In  this  case,  the  dentate 
nuclei  of  the  cerebellum  were  not  destroyed  as  in  Case  40, 
but  were  affected  by  cell  atrophies  of  variable  degree  in  differ- 
ent parts  of  the  nuclei.  There  was  also  a  severe  gliosis  of 
the  cerebellar  cortex.  The  left  hemisphere  of  the  cerebellum 
was  more  severely  diseased  than  the  right.  The  cortex 
showed  far  more  marked  and  generalized  cell  atrophies 
throughout  the  layers  than  did  Case  40.  The  details  of  this 
case,  which  was  that  of  a  colored  coachman,  Samuel  North, 
are  as  follows: 

He  was  born  in  1871.  Learned  to  read  and  write  at  school. 
Stableman  and  coachman.  Alcoholic  till  1902.  Took  much 
quinine,  possibly  impairing  hearing  thereby.  Memory  im- 
paired and  growing  worse  since  1902.  Gait  unsteady  for  a 
longer  but  unknown  period.  August  13,  1907,  wandered 
about,  instead  of  attending  boot-black  stand,  muttered, 
talked  incoherently.  In  the  next  few  days  talked  about 
religion  and  apparently  had  hallucinations  of  hearing.  Com- 
mitted August  1 6,  1907. 

On  commitment  stoop-shouldered,  flat-chested.  Gait  stag- 
gering. Unsteadiness  in  Romberg's  position.  Incoordina- 
tion  of  arms  and  fingers.  Coarse  tremor  of  tongue.  Tremor 
of  lower  jaw.  Exaggeration  of  left  knee-jerk  and  diminution 
of  right.  Exaggerated  Achilles  jerks.  Spurious  left  ankle 
clonus.  Questionable  Babinski  reaction  of  left  side.  Ab- 
dominal and  epigastric  reflexes  present  but  cremasteric 


PUZZLES   AND   ERRORS  173 

absent.  Left  pupil  smaller  than  right  and  fails  to  react  to 
light.  Reaction  of  right  pupil  sluggish.  Moderate  defect  of 
hearing  of  both  sides. 

During  the  first  week  the  patient  developed  hallucinations 
of  sight  and  hearing,  but  of  no  other  senses.  Disorientation 
for  time,  place,  and  persons.  Answers  to  arithmetical 
problems  given  with  assurance  but  as  a  rule  incorrectly  (as 
17  and  32  are  90;  18  divided  by  3  is  88).  Handwriting 
scarcely  legible.  Memory  poor,  especially  for  recent  events 
(recalled  a  lumbar  puncture  as  an  exercise  in  baptism). 
Impressibility  and  attention  poor.  Euphoria. 

Death  after  gradual  failure  July  29,  1908. 

Lumbar  puncture  showed :  Per  Cent. 

Endothelial  cells 9 

Lymphocytes 81 

Plasma  cells 6 

Phagocytes o 

Polymorphonuclear  cells 4 

Unclassified o 

Fibroblasts o 

Cells  in  100  fields 700 

Post  Mortem  Findings.  The  cerebrospinal  fluid  showed 
a  pure  culture  of  Bacillus  coli  communis,  and  the  heart's  blood 
showed  many  colonies  of  an  unidentified  bacillus.  Culture 
from  mesenteric  lymph  nodes  sterile. 

The  cause  of  death  is  somewhat  in  doubt.  There  was  an 
early  pneumonic  process  with  fibrinous  pleurisy,  and  there 
was  an  early  acute  hemorrhagic  ileitis  with  a  very  slight 
overlying  peritonitis  and  slight  corresponding  enlargement  of 
mesenteric  lymph  nodes.  There  was  an  infection  of  the 
meninges  with  Bacillus  coli  communis. 

Evidences  of  chronic  disease  outside  the  nervous  system, 
were:  coronary  and  pulmonary  arteriosclerosis,  chronic 
fibrous  endocarditis,  mitral  sclerosis,  aortic  sclerosis  with 
calcification,  chronic  splenitis,  chronic  interstitial  nephritis, 
hepatic  atrophy  (wt.,  900  grams),  thickening  of  cartilaginous 
portion  of  right  auricle  (old  trauma),  scars  of  apices  of  lungs. 

The  calvarium  was  dense  and  the  dura  mater  everywhere 
adherent.  The  arachnoidal  villi  were  but  slightly  developed, 


174 


PUZZLES   AND    ERRORS 


but  there  was  one  small  focus  of  cortical  herniation  through 
the  dura  mater  of  the  left  middle  cranial  fossa.  The  pia 
mater  was  delicate  except  for  slight  opacities  along  sulci. 
There  was  some  pial  thickening  over  the  region  of  the  inter- 
parietal  sulci  on  both  sides.  There  was  pial  pigmentation 
anteriorly  and  superiorly. 

There  is  no  gross  evidence  of  intracranial  arteriosclerosis, 
except  (i)  that  afforded  by  the  lesions  of  the  dentate  nuclei 
of  the  cerebellum  mentioned  below  and  (2)  the  swerving  to 
the  right  of  the  basilar  artery,  possibly  due  not  to  arterio- 
sclerotic  lengthening  of  the  artery  but  to  an  unusual  shape  of 
the  pons  (see  below). 

The  brain  weighed  1245  grams  (cerebellum  and  pons  165 
grams).  The  anatomical  diagnoses  of  central  nervous  system 
were: 

Slight  general  encephalomalacia  (post  mortem  imbibition 
of  fluid,  31  hours).  Slight  gliosis  of  right  prefrontal  and 
frontal  gyri.  Slight  gliosis  of  right  optic  thalamus.  General- 
ized granular  ependymitis,  especially  near  fornix  and  about 
foramina  of  Monro.  Anomaly  of  pons  (not  gliotic,  but 
possessing  far  more  white  matter  on  the  left  side  than  the 
right).  Severe  arteriosclerosis  confined  to  the  dentate  nuclei 
of  the  cerebellum. 

As  we  now  look  over  the  data  in  Cases  40  and  41  we  are 
inclined  to  ask  the  question,  whether  modern  systematic 
diagnosis  would  not  have  shown  these  cases  to  be  NEUROSYPHI- 
LITIC?  One  is  inclined  to  answer  this  question  in  the  affirma- 
tive, on  the  basis  that  Case  40  showed  somewhat  questionable 
Argyll- Robertson  pupils,  and  Case  41  showed  unilateral 
Argyll-Robertson  effect.  Both  cases  showed  Romberg  sign, 
but  the  dentate  nucleus  and  other  cerebellar  disease  in  each 
case  may  in  some  way  have  contributed  to  or  imitated  this 
phenomenon.  Whether  Case  40  was  a  tabetic  must  remain 
a  question,  but  Case  41  must  be  regarded  as  a  case  with  spinal 
and  meningeal  changes  highly  characteristic  of  syphilis. 


PUZZLES   AND   ERRORS  175 


VASCULAR  NEUROSYPHILIS  plus  TABETIC 
NEUROSYPHILIS  ("tabes  dorsalis  »)  simulating 
paretic  neurosyphilis  ("  general  paresis ").  Au- 
topsy. 


Case  42.  The  case  of  Elizabeth  Brown  was  at  one  time 
carefully  studied  by  Dr.  A.  M.  Barrett  in  his  work  on  mental 
diseases  associated  with  cerebral  arteriosclerosis  and,  like 
Case  43,  was  one  in  which  tabes  dorsalis  was  a  factor.  Eliza- 
beth Brown's  maternal  grandfather  and  mother  were  insane; 
there  had  also  been  insanity  in  a  sister.  Mrs.  Brown  was 
struck  on  the  head  at  44,  and  was  unconscious  for  an 
hour,  but  there  were  no  sequelae  to  this  accident.  At  48, 
there  was  a  shock,  or  apoplectiform  attack,  followed  by 
unconsciousness  for  two  hours  and  by  left  hemiplegia,  right 
ptosis,  and  thick  speech.  Mrs.  Brown  began  to  walk  again 
after  two  weeks,  but  was  found  to  be  forgetful  and  fabulatory. 
She  seemed  at  times  to  be  hearing  music,  and  somewhat 
repeatedly  became  helpless  and  unable  to  walk.  She  could 
not  remember  from  day  to  day,  showed  incontinence  of  urine 
and  feces,  and  was  brought  to  the  Danvers  Hospital.  The 
physical  and  mental  deterioration  was  progressive.  There 
were  some  signs  of  organic  brain  disease.  The  musculature 
was  especially  flabby  on  the  left  side.  The  left  angle  of  the 
mouth  drooped,  and  the  left  nasolabial  fold  was  smoothed 
out.  The  arm  movements  were  ataxic,  the  tongue  protruded 
to  the  left,  the  right  pupil  reacted  but  slightly  to  light  (eye 
blind  from  cataract),  the  knee-jerks,  Achilles,  wrist,  and 
elbow  reflexes,  were  absent.  The  patient  was  unable  to 
stand,  and  there  was  a  marked  tremor  of  the  hand,  tongue, 
and  lips.  There  was  a  zone  of  anesthesia  for  pain  and 
tactile  stimulation  extending  round  the  body,  from  the  3d 
to  the  6th  rib,  and  there  were  symmetrical  areas  of  anesthesia 
on  the  inner  surface  of  the  forearms  and  the  legs. 

The  autopsy  showed  a  general  arteriosclerosis  with  chronic 
and  acute  meningitis.  The  brain  weighed  mo  grams; 


176  PUZZLES  AND   ERRORS 

the  pia  mater  was  moderately  thickened;  the  basal  vessels 
were  highly  arteriosclerotic.  The  brain  itself,  however, 
normal  externally,  upon  dissection,  showed  a  number  of 
small  cysts  irregularly  scattered  in  the  white  substance. 
The  basal  ganglia  were  porous,  and  there  were  several  small 
cysts  in  the  pons.  Microscopically,  there  was  evidence  of 
severe  vascular  disease,  involving  not  only  the  arteries  but 
also  the  veins.  It  was  the  superficial  rather  than  the  deep 
arteries  that  were  more  often  attacked.  There  was  a  marked 
perivascular  gliosis.  Extensive  search  yielded  no  evidence 
of  lymphocyte  infiltrations,  either  in  the  brain  or  in  the  spinal 
cord. 

The  spinal  cord  showed  degenerations  in  both  the  lateral 
and  posterior  columns,  of  which  the  explanation  may  possibly 
be  like  that  in  our  paradigm,  Case  I. 

Is  the  case  of  Elizabeth  Brown  one  of  neurosyphilis?  We 
cannot  definitely  say  on  account  of  the  non-availability  of 
the  modern  systematic  tests,  but  it  may  well  be  that  the  case, 
although  certainly  not  one  of  paretic  neurosyphilis,  was  one 
of  TABES  WITH  VASCULAR  COMPLICATIONS. 


PUZZLES   AND    ERRORS  177 


TABETIC  NEUROSYPHILIS  ("tabes  dorsalis  ") 
with  symptoms  of  cerebral  origin  producing  a  pic- 
ture resembling  taboparetic  neurosyphilis  ("  tabo- 
paresis ").  Autopsy. 


Case  43.  Robert  Allen  was  the  fifth  case  of  error  in  the 
diagnosis  of  general  paresis  analyzed  some  years  since  from 
the  staff  meeting  records  of  the  Danvers  Hospital.  The 
Allen  case  resembles  the  case  of  Elizabeth  Brown  in  that 
there  was  a  combination  of  tabetic  phenomena  with  cerebral 
lesions  of  a  non-paretic  character  at  autopsy.  But  al- 
though there  seemed  to  be  an  utter  absence  of  inflammatory 
cells  (lymphocytosis)  in  the  case  of  Elizabeth  Brown  (42), 
there  were  some  slight  perivascular  cell  accumulations  in 
the  Allen  case,  with  a  few  mononuclear  cells  suggestive-  of 
lymphocytes.  The  cerebrum,  however,  failed  to  show  plas- 
mocytosis.  It  was  seriously  diseased,  showing  a  marked 
neuroglia  proliferation  about  the  atrophic  nerve  cells. 

Robert  Allen  was  a  printer  coming  from  a  long-lived  race. 
The  following  are  the  main  facts: 

Married  in  1875  (two  children,  healthy);  again  married  in 
1893  (one  child,  healthy).  Compositor  from  1890.  In  1898 
and  1899  girdle  and  lancinating  pains.  Thereafter  for 
several  years  gait  was  unsteady.  During  1904  and  1905 
freedom  from  pains  and  improvement  in  gait  but  gradually 
increasing  irritability  and  nervousness.  Stopped  work  on 
last  of  March,  1905,  owing  to  sudden  increase  of  irritability, 
emotionality,  boastfulness,  expansive  schemes,  and  ataxia. 

Habits:  no  tobacco,  very  little  alcohol  at  long  intervals. 
No  drug  habits,  no  sexual  irregularity  known. 

Committed  to  Danvers  April  3,  1905,  with  slight  muscular 
development,  poor  nutrition,  acne,  irregular,  poorly  preserved 
teeth,  gingivitis,  flat-foot,  slight  radial  arteriosclerosis,  slight 
arcus  senilis,  a  few  hyaline  casts,  leucocytes,  epithelial  cells, 
and  trace  of  albumin  in  the  urine,  scar  in  sulcus,  and  enlarged 
inguinal  lymph  nodes. 


PUZZLES  AND   ERRORS 

Ataxic  gait,  Romberg's  sign,  fibrillary  twitching  of  chest, 
abdominal  and  facial  muscles  when  standing;  right  pupil 
slightly  larger  than  left,  pupillary  margins  irregular,  light 
reactions  (electric  bulb  test)  both  consensual  and  direct 
absent,  slight  pupillary  reaction  in  accommodation;  biceps, 
triceps  and  wrist  reflexes  lively  and  equal;  abdominal,  cre- 
masteric  and  plantar  reflexes  normal,  knee-jerks,  Achilles 
and  front  taps  negative  even  on  reinforcement. 

The  patient  himself  stated  that  his  ataxia  began  in  1904, 
that  he  had  been  under  treatment  for  swelling  of  legs  and 
feet  and  pain  in  limbs  since  1903,  and  that  there  had  been 
some  trouble  with  limbs  since  1895.  He  had  been  told  that 
his  disease  was  lead-poisoning.  About  three  weeks  before 
commitment  patient  said  he  had  had  an  attack  of  uncon- 
sciousness. 

The  patient's  speech  showed  considerable  defect.  Words 
were  pronounced  slowly  with  slurring  and  tripping  especially 
of  the  labials.  Orientation  perfect.  School  knowledge  well 
retained.  The  easier  arithmetical  problems  were  accurately 
performed.  Memory  imperfect  for  minor  recent  events. 
Estimations  of  space  and  time  often  very  imperfect.  Vari- 
ability of  mood,  sometimes  euphoric,  sometimes  tearful  and 
irritable.  Occasional  expansive  estimates  of  personal  powers 
("Can  lift  three  five-hundred  pound  weights  with  one  finger"). 
Indistinct  expansive  financial  ideas. 

The  patient  continued  oriented,  euphoric,  expansive, 
untidy,  till  October,  1905,  but  on  October  12  developed  an 
infection  at  the  site  of  a  callus  on  the  sole  of  the  foot  and  died 
with  pyemic  symptoms,  October  17. 

Post  Mortem  Findings.  The  cause  of  death  was  strepto- 
coccus septicemia  with  acute  ulcerative  colitis,  acute  splenitis, 
bilateral  purulent  pleuritis,  multiple  infarctions  of  lungs. 

There  were  no  signs  of  chronic  disease  outside  the  nervous 
system  except  a  moderate  thickening  of  the  mitral  valves, 
and  slight  dural  adhesions. 

The  brain  weighed  1450  grams.  The  vessels  at  the  base 
showed  a  slight  degree  of  sclerosis.  There  was  a  slight 
opacity  of  the  frontal,  parietal,  and  temporal  pia  overlying 
slightly  atrophied  convolutions,  whose  surfaces  showed  in  a 


PUZZLES   AND   ERRORS  179 

few  places  slight  cuppings.  The  ependyma  over  the  thalami 
and  the  floor  of  the  fourth  ventricle  was  finely  roughened. 
The  spinal  cord  showed  a  typical  TABES  DORSALIS. 

Although  we  probably  cannot  regard  either  Case  42  or 
Case  43  as  a  case  of  paretic  neurosyphilis,  and  although  it 
must  remain  doubtful  whether  they  are  cases  of  any  form 
whatever  of  neurosyphilis  (in  the  absence  of  the  modern  tests), 
yet  it  seems  clear  that  both  these  cases  may  very  well  have 
been  cases  of  neurosyphilis  on  account  of  the  existence  of  a 
definite  tabetic  process  in  each.  The  symptoms  of  these 
cases,  like  those  of  Cases  38  to  41,  suggest  how  difficult  it 
must  be  to  make  a  clinical  diagnosis  of  general  paresis  safely 
without  employing  available  laboratory  tests.  Yet  how  fre- 
quently in  the  past  have  neurologists  brought  data  con- 
cerning various  phenomena  in  long  series  of  so-called 
paretics  in  which  the  error  of  diagnosis  was  certainly  between 
5  and  15%  and  frequently  still  greater.  The  entire  question 
of  the  symptomatology  of  paretic  and  non-paretic  neurosyphi- 
lis, therefore,  needs  re-opening  and  revision. 


PUZZLES  AND   ERRORS 


CEREBRAL  GLIOSIS  (probably  non-syphilitic) 
producing  the  clinical  picture  of  paretic  neurosyph- 
ilis  ("  general  paresis  ").  Autopsy. 


Case  44.  John  Hughes  was  a  hostler,  and  later  assistant 
with  a  wholesale  drug  company,  with  which  he  remained 
for  32  years.  He  had  been  moderately  but  constantly  al- 
coholic all  his  adult  life  up  to  50  years  of  age,  and  at  45  had 
had  an  attack  of  so-called  nervous  prostration,  in  which  his 
head  had  troubled  him  and  he  had  been  seclusive.  At  49, 
he  had  a  serious  attack  of  otitis  media,  associated  with 
delirium,  swelling  of  the  feet,  and  what  was  called  rheuma- 
tism. After  this  attack  of  otitis  media,  Hughes  appears  to 
have  been  not  altogether  right. 

At  53,  after  a  quarrel  with  his  employer,  Hughes  quit 
work,  began  to  trade  a  little  in  hens  and  pigs,  became  for- 
getful, especially  of  recent  events,  and  did  "  a  variety  of 
peculiar  things."  He  was  a  married  man  but  he  had  no 
children.  There  had  been  miscarriages  but  of  unknown 
origin;  venereal  disease  was  denied.  At  55,  a  week  before 
admission,  Hughes  had  a  spell  of  unconsciousness  for  several 
hours,  after  which  his  speech  was  thick,  and  restlessness,  in- 
somnia, and  a  wandering  tendency  set  in.  Visual  halluci- 
nations, fabulation,  tremors,  "  excited-looking "  eyes,  are 
described.  He  would  sweep  things  from  the  dining-room 
table,  pulled  a  hot  stove  into  the  middle  of  the  floor,  at- 
tempted to  sweep  paint  off  the  floor,  and  cut  up  a  carpet 
with  a  knife. 

The  patient  on  commitment  November  5,  1904,  was  well 
developed  and  nourished.  The  mucous  membranes  were 
rather  pale.  Bruises  and  excoriations  of  limbs.  Harsh 
breathing  at  the  base  of  each  lung.  Enlargement  of  heart; 
sounds  irregular.  Accentuation  of  aortic  second  sound; 
tension  fair,  rate  80.  Slight  brachial  arteriosclerosis.  Abdo- 
men slightly  distended.  The  urine  contained  a  faint  trace 
of  albumin  and  many  hyaline  casts. 


PUZZLES   AND    ERRORS  l8l 

Moderate  tremor  of  extended  hands.  Slight  tongue 
tremor.  Romberg's  sign  absent  (slight  swaying).  Con- 
siderable ataxia  of  extremities  (inability  to  stand  with  foot 
on  opposite  knee).  Vision  poor.  Hearing  could  not  be 
tested  accurately.  Prompt  pupil  reactions  with  direct  light. 
Slight  consensual  reaction  in  left  pupil,  absent  in  right.  Deep 
reflexes  equal  and  lively. 

Quiet  and  orderly  at  first.  Later  restless  and  noisy. 
Questions  were  answered  at  times  relevantly,  more  often 
irrelevantly.  Patient  irritable,  intractable.  Required  re- 
peated urging  to  take  nourishment.  Consciousness  clouded. 
Orientation  imperfect.  Attendants  are  possibly  "  officers." 
Date  September,  1995.  Slight  errors  in  repeating  alphabet. 
Mistakes  in  Lord's  Prayer  with  rhyming  tendency.  Simple 
arithmetical  tests  answered  automatically  with  many  mis- 
takes. More  complex  combinations  incorrect.  Handwriting 
tremulous  (noted  as  "  typical  of  general  paresis  ").  Auditory 
hallucinations  (answering  invisible  persons),  "All  right,  I'm 
coming."  Amnesia  and  confabulation.  Q.  "  Have  you 
had  breakfast?  "  A.  "  No,"  (later)  "  Yes,  I  had  a  very  light 
breakfast."  Q.  "What  did  you  have?"  A.  "Anything 
that  came  along.  A  few  green  peas  and  beans  that  were  left, 
bread  and  butter  and  pie.  I  had  a  good  breakfast.  Guess 
feed  is  very  high."  Q.  "  Give  names  of  your  sisters  and 
brothers."  A.  "  There  are  three  or  four  I  never  see.  I  will 
have  to  think  them  up."  (Later)  —  "  Lillie,  Abbie,  Julia, 
George."  On  repetition  of  question,  "  Elizabeth,  Julia, 
Annie  and  Lizzie.1' 

Delusions  somewhat  doubtful.     At  no  time  euphoria. 

The  patient  remained  only  nine  days  in  the  hospital, 
developing  diarrhea  a  week  after  admission. 

Post  Mortem  Findings.  The  cause  of  death  was  bilateral 
bronchopneumonia  of  hypostatic  distribution,  accompanied 
by  bronchitis  and  acute  splenitis.  The  intestinal  tract  was 
normal  (despite  the  diarrhea).  No  cultures.  The  heart 
showed  acute  myocarditis. 

The  vessels  in  general  showed  no  sclerosis,  except  that  the 
aorta  showed  a  few  patches  with  calcification  near  bifurcation. 
There  was  a  moderate  degree  of  mitral  sclerosis.  The  kidneys 


1 82  PUZZLES  AND   ERRORS 

showed  a  moderate  degree  of  chronic  interstitial  nephritis. 
The  heart  weighed  530  grams  and  there  was  moderate  dilata- 
tion of  all  the  valves. 

There  were  some  evidences  of  chronic  disease  outside  the 
nervous  system,  namely,  an  obliterative  pleuritis  on  the  right 
side,  chronic  perisplenitis,  and  chronic  external  adhesive 
pachymeningitis. 

The  nervous  system  showed  a  pia  mater  thin  and  trans- 
parent, with  a  moderate  congestion  of  larger  and  smaller 
vessels.  No  noteworthy  change  of  the  brain  substance  or  of 
the  ventricles  was  found,  except  that  the  cerebral  substance 
was  of  unusual  firmness  (autopsy  twelve  hours  after  death). 

It  is  clear  that  the  brain  was  not  wholly  normal,  exhibiting 
a  general  induration  due  in  part  to  subpial  gliosis  andjn  part 
doubtless  to  perivascular  gliosis.  Microscopically  the  tissues 
showed  features  of  great  interest,  especially  multiple  focal 
neuroglia  cell  proliferations  of  a  perivascular  distribution, 
considerable  subpial  fibrillar  gliosis  of  an  unusually  focal 
type,  and  a  rather  general  subpial  cellular  gliosis.  Histologi- 
cally,  it  seemed  that  this  chronic  progressive  process  had 
started,  not  so  much  in  relation  with  dying  nerve  cells,  as  in 
relation  with  blood  vessels.  The  perivascular  deposits  of 
neuroglia  cells  were  confined  almost  exclusively  to  the  in- 
fragranular  cortex  layers.  It  seems  plain  that  the  diagnosis  of 
general  paresis  was  not  justified.  It  is  probable  that  the  diag- 
nosis neurosyphilis  is  not  justified.  The  explanation  may 
be  that  now  and  then  cases  of  cerebral  sclerosis  may  clini- 
cally imitate  the  neurosyphilitic  process.  It  must  be  borne 
in  mind  that  the  diagnosis  in  this  case  was  made,  like  the  other 
cases  at  head  of  Part  III,  without  the  advantage  of  mod- 
ern systematic  methods.  Clinically  speaking,  of  course, 
there  was  no  definite  Argyll-Robertson  pupil,  although  the 
consensual  reaction,  slight  on  the  left  side,  was  absent  in  the 
right  pupil.  The  general  picture  appeared  to  be  one  of 
the  so-called  demented  form  of  paretic  neurosyphilis. 


PUZZLES   AND    ERRORS  183 


Differential  diagnosis  between  NEUROSYPHILIS 
and  NEURASTHENIA. 


Case  45.  Albert  Robinson,  a  man  of  28  years,  was  ship- 
wrecked on  one  of  the  Great  Lakes.  The  ship  was  on  the 
rocks  for  eight  days,  and  Robinson  was  under  a  great  strain. 
Ever  after  the  wreck,  Robinson  had  felt  severe  pain  in  the 
head,  neck,  and  back,  and  a  feeling  of  great  weakness  when- 
ever he  exerted  himself  physically  or  mentally,  and  seven 
months  after  the  wreck,  he  had  several  attacks  of  fainting. 

For  a  number  of  weeks  he  had  worried  a  good  deal  about 
his  inability  to  make  money,  especially  as  money  was  badly 
needed  on  account  of  his  wife's  approaching  confinement. 
A  few  days  before  entrance,  Robinson  had  become  very 
forgetful,  and  was  unable  to  recall,  the  night  before  entrance, 
where  he  had  been  during  the  day.  On  the  whole,  however, 
on  mental  examination  no  actual  evidence  of  memory  defect 
could  be  shown  to  exist. 

Physically,  Robinson  was  entirely  negative,  except  for 
some  hard  glands  in  each  groin.  Mentally,  there  was  little 
to  show  except  depression,  worry  over  his  financial  condi- 
tion, and  his  inability  to  work.  The  serum  W.  R.  proved 
negative. 

Diagnosis:  On  the  whole,  the  diagnosis  of  psychoneu- 
rosis  (see  case  Harrison  (9))  due  to  the  shock  at  the  time  of 
the  shipwreck  seemed  to  be  proper.  To  be  sure,  the  patient 
gave  a  history  of  a  chancre  at  25,  treated  for  two  years,  after 
which  he  was  declared  cured. 

However,  following  up  the  clue  of  admitted  syphilis, 
rigorous  questioning  elicited  the  fact  that  a  few  months 
before  there  had  been  diplopia,  lasting  part  of  a  day. 

Lumbar  puncture  seemed  desirable.  The  fluid  was  clear 
but  contained  125  cells  per  cmm.  with  appropriately  in- 
creased amounts  of  albumin  and  globulin.  The  spinal  fluid 
W.  R.  was  positive.  The  diagnosis  of  CEREBROSPINAL 
SYPHILIS  seemed  established. 


PUZZLES  AND   ERRORS 

The  lesson  of  this  case  appears  to  be  that  perhaps  we  should 
never  exclude  syphilis  until  we  have  made  an  examination 
of  the  cerebrospinal  fluid.  The  W.  R.  of  the  blood  in  meningo- 
vascular  (non-paretic  syphilis)  is  negative  in  many  cases 
(the  figure  is  sometimes  set  as  high  as  40%). 

Treatment:  After  a  half  dozen  injections  of  salvarsan, 
all  symptoms  disappeared  and  Robinson  went  back  to  work, 
claiming  to  be  in  a  better  condition  than  for  some  time  past. 

I.  How  shall  we  explain  such  a  symptom  as  the  transient 
diplopia?  This  diplopia  is  probably  an  example  of 
a  neurorecidive,  but  it  will  be  observed  that  it  occurred 
without  salvarsan  therapy.  See  discussion  above  under 
the  case  of  Bennett  (34),  where  the  general  result  of 
the  neurorecidive  inquiry  launched  by  Ehrlich  early 
in  the  history  of  salvarsan  therapy  showed  that  pre- 
cisely similar  phenomena  had  always  occurred  in 
neurosyphilis,  whether  under  treatment  or  not.  The 
anatomical  and  histopathological  explanation  of  such 
phenomena  is,  of  course,  doubtful,  but  a  review  of  the 
findings  in  the  case  of  Alice  Morton  (l)  will  show  how 
many  apparently  serious  symptoms  in  neurosyphilitics 
are  actually  irritative  or  at  least  due  to  lesions  which 
are  entirely  recoverable.  We  may  suppose,  first,  a 
local  proliferation  of  spirochetes;  second,  a  local  over- 
formation  of  toxic  substances,  directly  or  indirectly  the 
product  of  spirochetosis ;  thirdly,  a  local  exudation; 
fourthly,  a  local  proliferation;  fifthly,  a  combination 
of  these  phenomena,  any  or  all  of  which  may  be  regarded 
as  but  transient.  We  have  sometimes  found  at  autopsy 
very  little  exudate  except  in  small  areas;  sometimes 
not  more  than  a  few  mm.  or  cm.  in  superficial  extent. 
Note,  for  example,  the  small  areas  of  lymphocytosis 
demonstrable  in  but  two  foci  in  the  case  of  Alice 
Morton,  the  paradigm  placed,  at  the  beginning  of  this 
book. 


PUZZLES   AND   ERRORS  185 


NEUROSYPHILIS(?)  in  the  SECONDARY  STAGE 
of  syphilis.     HYSTERICAL  symptoms.    Diagnosis? 


Case  46.  Alice  Caperson  was  a  colored  girl  of  18  years. 
She  had  acquired  syphilis  five  months  before  admission  to 
the  hospital,  and  the  secondary  symptoms  of  this  syphilis 
had  just  disappeared  before  admission. 

Very  shortly  after  acquiring  syphilis,  the  young  negress 
began  to  act  peculiarly.  She  describes  herself  as  having 
a  sort  of  nightmare,  both  when  asleep  and  also  when  awake. 
For  instance,  she  saw  her  dead  grandmother.  It  appeared 
at  first  like  a  seraph;  then  it  came  nearer  to  her  and  seemed 
to  fill  out ;  and  then  was  dressed  precisely  as  her  grandmother 
had  been.  This  seraph  appeared  as  though  trying  to  tell  her 
something,  but  she  could  not  make  out  what  the  something 
was.  The  vision  had  appeared  on  two  or  three  occasions. 

Our  examination  detected  little  beyond  instability  and 
irritability  of  mood  with  some  depression.  The  patient 
readily  fell  to  weeping.  She  soon  made  friends  in  the  wards, 
however,  and  got  on  well.  Physical  examination  was  en- 
tirely negative  but  the  W.  R.  of  the  blood  serum  was  positive. 
The  W.  R.  of  the  spinal  fluid  was  negative,  as  was  the  gold 
sol  reaction;  there  was  an  excess  of  albumin  and  a  positive 
globulin  test;  there  were  seven  cells  per  cmm. 

The  psychiatric  diagnosis  of  a  case  like  that  of  Alice  Caper- 
son  would  waver  between  hysteria  and  dementia  praecox. 
However,  as  for  dementia  praecox  there  are  hardly  any 
typical  symptoms.  There  is  insight  into  the  hallucinations, 
which  are  hypnagogic.  There  are,  however,  no  hysterical 
stigmata. 

The  spinal  fluid  reaction  is  typical  of  the  secondary  stage 
of  syphilis.  It  is  commonly  said  that  in  every  case  of  syph- 
ilis the  nervous  system  is  involved  at  some  period,  if  only 
to  the  degree  shown  in  the  present  case.  However,  such 
involvement  tends  to  disappear  both  with  and  without  anti- 
syphilitic  treatment,  just  as  do  the  secondary  skin  symptoms. 


1 86  PUZZLES   AND   ERRORS 

So  far  as  syphilis  is  concerned,  the  prognosis  under  radical 
treatment  is  as  good  as  usual.  We  are  inclined  to  regard 
the  case  as  one  of  the  HYSTERICAL  or  PSYCHOPATHIC  group 
and  inasmuch  as  cases  occurring  in  the  developmental  stage 
of  a  patient's  life  are  of  fairly  good  general  prognosis,  we 
are  inclined  to  regard  the  prognosis  in  this  particular  case  as 
good  under  proper  therapy  and  hygiene. 

1.  What  is  the  relation  of  neuroses  to  syphilis?     Neuras- 

thenia, chorea,  hysteria,  and  epilepsy  are  often  grouped 
(for  example,  by  Nonne)  as  neuroses  bearing  at  times 
important  relations  to  neurosyphilis.  (For  the  relations 
of  neurasthenia,  chorea,  and  epilepsy,  see  cases  of 
Greeley  Harrison  (9),  Margaret  Green  (72),  and  David 
Borofski  (49),  respectively.)  As  for  the  hysteria  shown 
in  Caperson,  Charcot  enumerated  syphilis  among  agents 
provocateurs  of  hysteria  along  with  alcohol,  lead,  ar- 
senic, and  the  like.  Fournier  has  also  considered  the 
problem.  It  is  clearly  necessary  to  show  that  before 
infection  there  were  no  hysterical  symptoms,  and  that 
the  hysteria  developed  during  the  operation  of  the  syph- 
ilitic process,  and  it  is  probably  necessary  to  show  that 
the  symptoms  will  clear  up  under  antisyphilitic  treat- 
ment, if  we  are  to  concede  the  existence  of  a  syphilitic 
hysteria. 

2.  What  are  the  evidences  of  neurosyphilis  in  the  secondary 

and  primary  stages  of  syphilis?  As  above  stated,  the 
findings  in  Caperson  are  typical  enough.  Wile  and 
Stokes  at  first  stated  that  60  to  70%  of  the  secondary 
syphilitics  show  changes  in  the  spinal  fluid;  in  a  fur- 
ther article  they  maintain  that  probably  every  case 
shows  such  changes  and  that  clinical  symptoms  of 
neurosyphilis  of  the  secondary  period  can  probably  be 
determined.  They  claim  that  it  is  probable  also  that 
the  same  holds  for  primary  syphilis  itself.  The  im- 
portance of  these  claims  lodges  partly  in  the  relation 
of  these  early  signs  of  neurosyphilis  to  the  whole  ques- 
tion of  latency  and  to  the  question  of  paresis  sine  paresi. 
For  a  discussion  of  paresis  sine  paresi  see  cases  Lawlor 
(25),  Vogel  (52). 


PUZZLES  AND   ERRORS  187 


Differential  diagnosis  between  NEUROSYPHILIS 
and  MANIC-DEPRESSIVE  PSYCHOSIS.* 


Case  47.  As  in  other  instances  (compare  Martha  Bartlett 
(21)  and  Annie  Monks  (85))  so  also  in  the  case  of  Ethel  Hunter, 
a  woman  61  years  of  age,  there  was  no  initial  suspicion  of 
neurosyphilis.  Mrs.  Hunter  was  brought  to  the  hospital 
stuporous  as  a  result  of  an  overdose  of  paraldehyd.  The 
paraldehyd  had  been  administered  by  a  physician  to  combat 
insomnia  and  agitation.  As  soon  as  Mrs.  H.  had  recovered 
from  the  drug  stupor,  this  agitation  appeared  once  more,  and 
it  was  clear  that  she  was  suffering  from  marked  depression. 
There  was  tremendous  worry  over  the  sickness  of  a  woman 
with  whom  the  patient  lived.  The  patient  was  very  self- 
accusatory,  blaming  herself  for  many  things  that  had  hap- 
pened in  the  household.  Besides  her  agitation,  depression, 
self -accusations,  and  insomnia,  the  patient  showed  a  good 
deal  of  the  symptom  frequently  termed  "  retardation  "  — 
a  kind  of  lagging  of  all  mental  processes  found,  according  to 
Kraepelin,  in  manic-depressive  psychosis. 

Accordingly,  the  diagnosis  of  manic-depressive  psychosis 
might  well  have  been  rendered.  The  fact  that  the  psychosis 
so  far  as  known  began  in  the  involution  period  was  not  against 
the  diagnosis  since  the  so-called  involution-melancholia  of 
this  period  is  at  least  in  a  certain  fraction  of  cases  nothing 
more  or  less  than  a  form  of  manic-depressive  psychosis. 
However,  the  physical  examination  made  the  diagnosis  of 
manic-depressive  psychosis  a  little  doubtful.  There  was  a 
superficial  thickening  of  the  arteries  (blood  pressure:  systolic, 
170;  diastolic,  104),  which  thickening  would  not  in  itself  be 
against  the  diagnosis  of  manic-depressive  psychosis.  (In 
point  of  fact,  arteriosclerosis  is  rather  common  late  in  this 

*  A.  M.  Barrett  has  recently  discussed  this  subject  in  a 
paper  in  the  Journal  of  the  American  Medical  Association, 
Vol.  LXVII,  Dec.  2,  1916. 


PUZZLES  AND   ERRORS 

disease  and  previous  attacks  could  not  be  excluded  on  the 
basis  of  available  history.)  The  contracted  pupils  were 
irregular  and  both  reacted  sluggishly  to  light,  although  better 
to  accommodation;  the  right  pupil  was  larger  than  the  left. 
The  arm  reflexes  were  pretty  active.  The  left  knee-jerk 
could  not  be  obtained,  nor  was  the  right  knee-jerk  more  than 
very  sluggish.  The  Achilles  reflexes  could  not  be  obtained. 
Although  there  was  not  a  positive  Romberg  sign,  there  was 
a  considerable  swaying  in  Romberg  position.  There  was 
no  speech  defect.  The  other  reflexes  showed  nothing  ab- 
normal. On  the  whole,  we  had  to  conclude  that,  although 
Mrs.  Hunter  might  be  an  instance  of  manic-depressive  psy- 
chosis, still  there  was  much  of  neurological  interest  in  the 
case. 

This  conclusion  was  emphasized  when  the  W.  R.  of  the 
blood  serum  was  found  to  be  positive.  The  spinal  fluid  W.  R. 
was  also  positive,  and  the  gold  sol  index  was  of  the  "  paretic  " 
type.  There  were  74  cells  to  the  cmm.  Globulin  stood  at 
+!+++,  and  albumin  at  +  +  ++. 

This  case,  therefore,  again  illustrates,  as  well  the  protean 
nature  of  GENERAL  PARESIS  (the  diagnosis  rendered),  as  the 
doubtful  value  of  making  a  psychiatric  diagnosis  without 
due  consideration  of  the  physical  examination  and  laboratory 
findings.  How  easy  might  it  have  been,  at  least  some  years 
ago,  to  consider  that  this  patient  of  61  years  had  suffered  a 
slight  shock  at  some  previous  time  (left  knee-jerk  absent), 
but  was  as  a  matter  of  fact  a  case  of  manic-depressive 
psychosis  with  a  vascular  complication ! 

Note :  We  must  again  duly  insist  that  the  merely  sluggish 
light  reactions  of  the  pupils  in  such  a  case  as  this  do  not 
especially  point  to  general  paresis.  The  literature  seems  to 
establish  that  sluggishness  of  light  reaction  precedes  the 
classical  Argyll-Robertson  pupil.  Yet  it  does  not  do  to  say 
that,  if  the  Argyll-Robertson  pupil  pretty  conclusively  points 
to  neurosyphilis  (for  exceptions  see  cases  Falvey  (55),  Murphy 
(60)),  then  a  sluggish  pupillary  reaction  to  light  looks  in  the 
same  direction.  Sluggishness  may  precede  stiffness  in  many, 
or  perhaps  all,  cases,  but  sluggishness  of  pupils  is  a  frequent 
phenomenon  outside  the  syphilitic  group  of  cases. 


PUZZLES  AND  ERRORS  189 

1.  What  part  is  played  by  emotional  shock  and  psychic 

causes  in  the  starting  up  of  general  paresis?  The 
answer  to  this  question  cannot  be  definite.  That  a 
paretic  process  can  be  started  up  after  trauma  is  ad- 
mitted on  all  sides;  but  we  here  suppose  actual  physi- 
cal or  chemical  brain  disturbance  permitting  increased 
spirochetosis  or  inflammatory  reaction.  In  the  case  of 
psychic  shock,  or  what  might  be  called  psychogenic 
general  paresis,  our  best  resort  will  be  to  the  indirect 
effects  of  hormone  action,  or  of  vasomotor  and  other 
autonomic  disturbances  produced  directly  or  indirectly 
by  emotion.  We  are  clearly  here  dealing  with  material 
too  speculative  to  be  of  practical  service  at  this  time. 

2.  Was  the  depressive  drug  therapy  in  the  case  of  Hunter 

justifiable?  The  paraldehyd  had  been  administered  by 
a  physician  apparently  on  purely  symptomatic  grounds 
to  combat  the  insomnia  and  agitation  of  this  woman  of 
6 1  years.  With  all  due  acknowledgment  of  the  diffi- 
culties of  private  practice,  we  must  insist  that  when 
ordinary  measures  in  the  relief  of  insomnia  and  agitation 
are  insufficient  to  curb  these  conditions,  then  a  positive 
danger  ensues  with  the  larger  doses.  As  a  rule,  with 
these  larger  doses  and  with  the  withdrawal  of  sen- 
sory stimulation,  the  patients  relapse  into  a  stupor  of 
grave  moment.  We  need  only  recall  the  situation  in 
delirium  tremens  where  adequately  depressive  drugs 
often  tend  to  kill  the  patient. 


190 


PUZZLES   AND   ERRORS 


Case  for  diagnosis.  Errors  in  the  diagnosis  of 
NEURO SYPHILIS  are  possible  even  when  abun- 
dant clinical  and  laboratory  data  are  available. 


Case  48.  The  first  error  chosen  for  demonstration  is  that 
in  the  case  of  the  machinist,  Milton  Safsky. 

Safsky,  about  8  months  before  his  entrance  to  the  hospital 
in  the  42d  year  of  his  life,  had  begun  to  lose  strength,  to  grow 
thin  and  pale,  and  to  suffer  from  an  extreme  and  continuous 
thirst.  He  was  said  to  have  drunk  as  much  as  6|  gal.  in  a  day, 
and  passed  appropriately  large  quantities  of  urine.  After  a 
time,  his  management  at  a  general  hospital  became  difficult, 
as  Safsky  became  confused,  cried  "  hysterically,"  and  was  at 
times  very  noisy.  He  sustained  a  marked  memory  loss, 
seemed  to  show  visual  hallucinations,  and  complained  of 
headache,  both  frontal  and  occipital,  and  of  pain  about  the 
eyes.  Sometimes  the  patient  was  very  euphoric  and  ex- 
pressed what  seemed  to  be  delusions  of  grandeur,  saying  he 
was  wealthy  and  qwned.  many  machine  shops. 

Some  symptoms,  e.g.,  polydipsia  and  polyuria  amounting 
to  a  diabetes  insipidus,  associated  with  headache  and  arrested 
attention,  suggested  possibly  a  new  growth  in  the  pituitary 
region.  The  mental  symptoms  might  naturally  be  supposed 
to  be  due  to  some  infiltration  or  pressure  effect  of  intracranial 
growth.  After  admission  to  the  Psychopathic  Hospital,  the 
patient  was  found  difficult  to  arouse,  although  he  could 
eventually  be  aroused.  His  orientation  proved  to  be  as  poor 
as  his  memory.  From  time  to  time,  the  patient  became  a 
bit  more  intelligent  and  able  to  execute  requests. 

The  physical  examination  was  in  general  almost  entirely 
negative.  Neurologically,  the  pupils  were  markedly  con- 
tracted and  reacted  slowly  to  light,  though  they  were  other- 
wise normal.  The  deep  reflexes  were  all  somewhat  lively, 
though  equal.  The  umbilical  and  cremasteric  reflexes  in 
particular  were  present.  Systematic  examination  revealed 
no  other  reflex  disorder,  nor  any  disturbance  of  sensation. 


PUZZLES  AND   ERRORS  19! 

There  was  a  coarse  tremor  of  the  extended  hands.  There 
were  no  phenomena  of  importance  in  the  visual  fields. 

As  against  the  diagnosis  of  growth,  pituitary  or  extra- 
pituitary  (diabetes  insipidus  and  headache),  a  hypothesis  of 
neurosyphilis  had  to  be  considered.  Not  only  were  the  con- 
tracted, slowly-reacting  pupils  and  the  active  deep  reflexes 
suggestive,  but  the  euphoria  with  grandiose  ideas  looked 
entirely  consistent.  As  for  the  polyuria,  one  had  to  think  of 
the  so-called  syphilitic  polyuria  of  the  textbooks,  which  is 
regarded  as  a  more  or  less  characteristic  result  of  syphilitic 
involvement  of  the  basis  cerebri.  Moreover,  the  W.  R.  in 
the  spinal  fluid  proved  to  be  slightly  positive;  146  cells  per 
cmm.  were  found  therein;  there  was  a  large  quantity  of 
globulin,  and  a  very  marked  increase  in  albumin.  These 
observations  seemed  to  be  exceedingly  suggestive  of  a  cerebral 
syphilis. 

However,  as  the  case  progressed,  the  diagnostic  situation 
changed.  The  W.  R.  upon  a  second  puncture  fluid  proved 
negative.  After  some  weeks,  characteristic  symptoms  of  in- 
tracranial  pressure  developed ;  the  diagnosis  of  BRAIN  TUMOR 
had  to  be  taken  as  established,  and  there  is  no  doubt  of  its 
correctness. 

1.  What  is  the  explanation  of  the  weakly  positive  W.  R.  in 

Safsky's  spinal  fluid?  An  explanation  is  not  easy  to 
find.  Possibly  we  may  regard  the  reaction  as  an  ex- 
ample of  error  in  technique.  It  is  even  possible  that  it 
may  have  been  produced  by  exudative  products  in  the 
spinal  fluid. 

2.  What  precautions  may  be  taken  against  an  error  in 

diagnosis  such  as  was  first  made  through  the  positive 
spinal  fluid  Wassermann  in  the  case  of  Safsky?  First, 
repetition  of  the  W.  R.;  secondly,  it  is  very  unusual 
to  find  a  weakly  positive  W.  R.  in  a  case  with  such 
marked  excess  of  albumin  and  such  very  marked  in- 
crease of  globulin  as  was  shown  by  this  case. 

3.  How  can  we  explain  the  inflammatory  products  in  the 

puncture  fluid?  Superficial  brain  tumors  are  fre- 
quently associated  with  a  so-called  meningitis  sym- 
pathica.  The  products  of  such  meningitis  are  ex- 
hibited: viz.,  globulin,  albumin,  and  pleocytosis,  exactly 
as  shown  in  Safsky. 


PUZZLES  AND   ERRORS 


Can  PARETIC  NEUROSYPHILIS  ("general 
paresis  ")  appear  clinically  EARLY  (e.g.,  two  years) 
after  the  initial  syphilitic  infection? 


Case  49.  David  Borofski,  a  street  car  conductor,  27  years 
of  age,  suddenly  had  a  convulsion  while  at  work  in  his  car. 
For  four  months  Borofski  continued  to  have  rather  numerous 
convulsions,  was  finally  compelled  to  discontinue  work,  and 
resorted  to  the  Psychopathic  Hospital.  It  appears  from  his 
own  story  that,  about  two  years  before,  he  had  had  a  chancre, 
for  which  he  had  been  treated  at  a  general  hospital  syphilis 
clinic,  and  of  which  he  was  told  he  was  cured.  With  a  pro- 
gressive loss  of  memory  and  with  convulsions,  Borofski  be- 
came much  concerned  about  himself,  and  was  finally  per- 
suaded by  his  fellow-workers  to  come  to  the  Psychopathic 
Hospital. 

The  convulsions  were  described  as  follows:  The  patient 
gives  a  short  cry,  has  convulsive  movements  for  about  ten 
minutes,  remains  unconscious  for  perhaps  half  an  hour,  and 
wakes  with  headache,  dizziness,  and  a  feverish  appearance. 
Sometimes  the  attacks  were  more  severe,  with  frothing  at 
the  mouth,  biting  of  lips,  and  loss  of  sphincter  control.  There 
were  also  slight  attacks,  occurring  almost  every  day,  without 
loss  of  consciousness;  these  latter  attacks  consisted  of  diz- 
ziness, inability  to  speak  for  a  few  seconds,  and  some  arm 
twitching. 

Physically,  Borofski  was  well  developed  and  nourished,  with 
a  blood  pressure  of  160.  The  only  abnormal  phenomena 
neurologically  were  absent  knee-jerks  and  ankle-jerks,  slug- 
gish pupillary  reactions,  and  slight  tremor  of  the  hands. 

Mentally,  despite  suggestive  complaint  of  amnesia,  the 
memory  was  found  to  be  fairly  good  but  knowledge  of  cur- 
rent events  and  school  knowledge  was  poor.  The  simplest 
problems  in  arithmetic  Borofski  gave  up. 

The  first  diagnosis  in  such  a  case  would  naturally  be 
epilepsy.  However,  when  an  epileptic  or  epileptiform  at- 


PUZZLES  AND   ERRORS  193 

tack  occurs  for  the  first  time  in  adult  life,  the  chances  are 
probably  against  an  idiopathic  epilepsy.  (This  is  not  a 
universal  rule  but  will  serve.)  Borofski  himself,  moreover, 
gave  a  history  of  syphilis.  And  the  very  nature  of  the  attacks, 
with  arm  twitching  and  without  loss  of  consciousness,  would 
not  readily  fit  into  the  frame  of  the  idiopathic  group.  The 
absence  of  certain  reflexes  and  the  sluggish  pupils  are  naturally 
also  suggestive  of  syphilis,  although  not  convincing. 

The  W.  R.  of  the  serum  proved  positive,  as  did  that  of 
the  spinal  fluid.  The  gold  sol  reaction  was  characteristically 
"paretic";  there  was  an  excess  of  albumin  and  a  positive 
globulin,  and  there  were  15  cells  per  cmm.  There  could  be 
little  or  no  doubt  of  the  diagnosis  of  some  form  of  neuro- 
syphilis.  The  laboratory  picture  was  consistent  either  with 
general  paresis  or  with  cerebrospinal  syphilis.  So  far  as  we 
are  aware  in  the  present  stage  of  knowledge,  the  two  conditions 
can  hardly  be  differentiated  unless  we  choose  to  rely  on 
therapeutics.  However,  it  is  exceedingly  rare  for  general 
paresis  to  occur  only  two  years  after  the  original  infection. 
If  we  can  trust  this  statistical  fact,  we  shall  perhaps  be  wiser 
to  term  the  case  of  Borofski  one  of  DIFFUSE  CEREBROSPINAL 
SYPHILIS,  and  not  one  of  paresis. 

Treatment:  Borofski  was  put  on  antisyphilitic  treat- 
ment consisting  of  0.6  gram  of  salvarsan  twice  a  week  and 
potassium  iodid,  together  with  intramuscular  injections  of 
mercury  salicylate.  The  convulsions  then  ceased.  After 
four  months  Borofski  returned  to  work,  and  he  has  remained 
at  work  for  a  year.  He  has  never  regained  his  former  health. 

Fifteen  months  after  beginning  of  treatment  the  laboratory 
tests  were  again  made  (there  had  been  more  than  60  injec- 
tions of  salvarsan),  and  the  cell  count  and  gold  sol  reactions 
were  found  to  be  negative.  Globulin  and  albumin  were 
also  in  smaller  amounts  than  in  the  original  examination. 
However,  the  W.  R.  of  the  serum  and  the  spinal  fluid  remained 
positive. 

Head  and  Fearnsides  state  that  cases  of  cerebrospinal 
syphilis  should  return  negative  spinal  fluid  tests  after  six 
months  of  treatment.  Upon  this  criterion  of  Head  and 
Fearnsides,  Borofski  would  not  be  a  case  of  cerebrospinal 


194  PUZZLES  AND   ERRORS 

syphilis;    but  it  is  probably  impossible  to  separate  various 
forms  of  neurosyphilis  into  categories  on  any  such  grounds. 

1.  Shall  case  David  Borofski  be  regarded  as  one  of  paretic 

neurosyphilis  ("general  paresis")  ?  He  has  returned  to 
work  and  has  remained  at  work,  though  without  regain- 
ing his  former  health.  In  any  event,  however,  he  does 
not  offer  the  typical  picture  of  inevitable  decline  and 
death  presented  by  the  typical  case  of  Pietro  Martiro 
(15)  presented  in  our  discussion  of  systematic  diag- 
nosis. However,  we  could  not  upon  laboratory  grounds, 
or  even  upon  the  ground  of  clinical  observation,  dis- 
tinguish Borofski  from  Martiro;  Borofski  has  greatly 
improved;  Martiro  is  dead.  Borofski  developed  his 
obvious  neurosyphilis  only  two  years  after  the  original 
infection.  The  conservative  syphilographer  might,  ac- 
cordingly, reply  that  David  Borofski  is  not  a  typical 
case  of  paretic  neurosyphilis  ("general  paresis")  either 
in  the  length  of  the  incubation  period  for  his  neuro- 
syphilitic  symptoms,  or  in  his  outcome. 

2.  What  is  the  cause  of  such  convulsions  as  those  developed 

by  David  Borofski?  Evidence  from  clear  cases  of 
general  paresis  with  convulsions  leads  to  the  hypothesis 
that  such  convulsions  as  those  developed  by  Borofski 
are  not  necessarily  based  upon  frank  destructive 
lesions  such  as  would  be  produced  by  the  plugging  of 
terminal  arteries.  They  may  well  be  produced  through 
the  activities  of  minor  lesions,  only  demonstrable  by 
microscopic  methods,  either  through  properly  disposed 
cell  losses  or  by  the  pressure  of  exudate,  or  even  by 
endotoxins  or  other  substances  derived  from  the  bodies 
of  dead  or  living  spirochetes. 

3.  Aside  from  the  well-known  syphilitic  epilepsy  due  to 

meningitis,  is  there  a  non-meningitic  epilepsy  (such 
a  disease  as  Fournier  formerly  described  under  the  term 
parasyphilitic  epilepsy)?  We  dismiss  from  discussion 
the  so-called  symptomatic  epilepsies  which  are  the 
result  of  a  gross  organic  disease  of  the  brain  substance 
or  its  membranes,  and  which  do  not  differ  so  far  as  we 
are  aware  from  organic  epilepsy  produced  by  other 
gross  lesions  of  an  identical  size  and  structure.  These 
symptomatic  epilepsies  may  be  partial,  or  even  may 
present  the  appearance  of  generalized  epilepsy.  We 
may  also  leave  out  of  account  those  epileptic  pictures 
which  are  produced  in  general  paresis  itself,  and  which 


PUZZLES   AND   ERRORS  195 

may  be  viewed  as  nothing  but  partial  phenomena  of 
general  paresis.  The  kind  of  so-called  "  parasyphilitic  " 
epilepsy  that  Fournier  described  is  a  kind  of  epilepsy 
that  cannot  be  distinguished  from  genuine  epilepsy,  in 
which  the  sole  disease-phenomenon  throughout  a  long 
period  of  time  consists  of  epileptic  convulsions.  It  ap- 
pears that  these  "  parasyphilitic  "  imitations  of  genuine 
epilepsy  occur  in  individuals  with  a  very  long  post- 
infective  "  incubation  period,"  but  that  there  are  some 
cases  in  which  the  epilepsy  appears,  on  the  contrary, 
in  the  very  earliest  stages  of  syphilis.  The  attacks 
are  a  little  less  common  than  those  of  idiopathic  epi- 
lepsy; they  have  the  same  apparently  causeless  be- 
ginning; are  associated  with  complete  amnesia;  and 
are  followed  by  characteristic  dazed  states.  The 
patient's  intelligence,  however,  suffers  little.  Now  and 
then  a  case  reacts  well  to  antisyphilitic  treatment  ener- 
getically pushed.  (Spontaneous  long  remissions  in 
non-syphilitic  epilepsy  must  be  remembered.)  Petit 
mal  attacks  occur  sometimes  between  the  more  severe 
attacks.  In  short,  it  would  appear  that  there  is  a 
group  of  syphilitic  epilepsies  in  which  the  brain  shows 
no  gross  structural  lesions,  which  accordingly  do  not 
exhibit  any  Jacksonian  appearances,  and  which  last 
a  comparatively  long  time  without  changing  their 
character,  and  often  without  being  especially  altered 
for  the  better  by  any  form  of  antisyphilitic  treatment. 
This  condition  is  sometimes  known  as  a  post-syphilitic 
epileptic  neurosis.  Nonne  had  been  able  to  collect  up 
to  1902  some  12  cases  from  his  own  service. 

4.  Would  it  be  proper  to  call  Borofski  a  case  of  taboparesis? 

Absent  knee-jerks  in  a  victim  of  paretic  neurosyphilis 
should  not  be  used  to  suggest  a  diagnosis  of  taboparesis. 
This  question  of  terminology  has  been  discussed  above, 
under  Sullivan  (16). 

5.  What  is  the  mechanism  by  which  the  amnesia  of  a  case 

like  Borofski  is  produced?  The  answer  runs  in  the 
same  terms  as  the  answer  to  the  questions  concerning 
the  cause  of  convulsions.  The  amnesia  in  general 
paresis  has  surprising  functionality.  A  study  of  autop- 
sied  cases  of  general  paresis  has  shown  that  amnesia 
is  practically  as  common  in  cases  without  marked 
destruction  of  brain  tissue  as  in  cases  with  atrophy  of 
classical  extent  and  depth.  The  clinical  recovery  in 
this  case  was  practically  complete  in  respect  to  memory. 
We  must  regard  the  amnesia  as  not  due  to  the  destruc- 


196  PUZZLES  AND  ERRORS 

tion  of  storage  cells  bearing  the  so-called  neurograms 
(Morton  Prince). 

6.  What  is  the  explanation  of  the  persistently  positive  W. 
R.'s  of  the  serum  and  spinal  fluid  associated  with 
diminished  globulin  and  albumin  tests,  a  negative  gold 
sol  reaction,  and  normal  cell  count?  See  discussion 
under  Case  Martha  Bartlett  (21). 

How  atypical  is  the  early  development  of  paretic  symp- 
toms in  David  Borofski?  C.  B.  Craig  has  collected, 
in  loo  cases  of  brain  syphilis  (a  list  including  both 
paretic  and  non-paretic  cases),  some  data  on  this 
point.  The  shortest  period  reported  by  Craig  was  in 
a  case  in  which  the  neurosyphilitic  symptoms  appeared 
one  month  after  infection.  Craig  found  three  cases 
where  symptoms  appeared  in  six  months,  and  six  cases 
within  a  year.  The  longest  post-infective  period  of 
Craig's  list  was  thirty  years.  Our  case  of  Chatterton 
(73)  developed  symptoms  33  years  after  infection  and 
Washington  (66),  forty  years  after  infection.  Nonne 
casts  some  doubt  on  statements  to  the  effect  that 
tabetic  symptoms  may  occur  three  to  four  months 
after  infection.  It  seems  to  be  admitted  that  pupillary 
anomalies  and  reflex  changes  may  occur  in  the  early 
secondaries  and  may  recover  under  antisyphilitic  treat- 
ment. Nonne's  case  of  longest  post-infective  interval, 
like  that  of  Craig,  was  one  of  30  years. 

Myerson  has  reported  a  2O-year  old  patient  who 
acquired  chancre  April  I,  1911  (spirochetes  demon- 
strated) ;  salvarsan  was  administered  April  2oth.  There 
were  no  secondary  symptoms,  but  in  May,  headache, 
visual  disturbance,  vertigo,  and  other  symptoms  de- 
veloped (neurorecidive).  Upon  June  2Oth,  that  is, 
II  weeks  after  development  of  the  chancre,  aphasia 
and  astasia  developed,  with  numbness  of  the  left  side. 
At  this  time,  the  pupils  were  slightly  irregular  and  un- 
equal but  reacted  normally.  The  signs  in  the  fluid 
were  positive.  Upon  this  question  see  our  cases  of 
Bright  (121)  and  Bennett  (34). 


PUZZLES   AND   ERRORS  197 


HEMITREMOR  following  hemiplegia  in  PARETIC 
NEUROSYPHILIS  ("  general  paresis  ")•    Autopsy. 


Case  50.  Achilles  Akropovlos,  39  years,  had  symptoms 
six  months  before  commitment  to  Danvers  Hospital.  There 
were  attacks  of  confusion,  difficulty  in  walking,  and  speech 
defect,  resulting  in  an  entire  incapacity  to  work  and 
eventual  commitment.  Rather  unusual  and  striking  was  a 
very  marked  tremor,  apparently  limited  to  the  right  side  of 
the  body.  Physically,  Akropovlos  was  normal,  but  neuro- 
logically  he  showed,  in  addition  to  the  marked  right-sided 
tremor,  a  marked  speech  defect,  and  a  degree  of  ataxia. 
The  tendon  reflexes  were  very  active,  but  there  were  no  ab- 
normal reflexes,  and  the  pupils  reacted  normally.  According 
to  the  history,  the  difficulty  had  followed  a  slight  attack  of 
apoplexy.  Mentally,  there  was  a  marked  confusion.  The 
blood  serum  and  the  spinal  fluid  were  both  positive  to  the  W. 
R. ;  globulin  was  present,  and  albumin  was  increased;  there 
were  43  cells  per  cmm.  There  was  hardly  any  diagnosis  to 
make  except  general  paresis. 

Death  followed  18  months  later,  or  two  years  after  onset 
of  symptoms.  Increasing  weakness,  emaciation,  and  de- 
mentia preceded  death.  Autopsy  confirmed  the  diagnosis  of 
PARETIC  NEUROSYPHILIS. 

I.  What  is  the  usual  cause  of  death  in  general  paresis? 
Intercurrent  disease  very  frequently  occurs  in  general 
paresis,  and  such  intercurrent  disease  is  then  given  as 
the  cause  of  death.  As  a  matter  of  fact,  however,  one 
feels  that  in  many  of  these  cases  the  intercurrent 
pneumonia  or  infection  —  frequently  of  the  bladder, 
-bedsores,  sepsis,  and  the  like,  are  merely  accidental 
incidents  in  a  condition  that  is  leading  to  death,  and 
which  has  caused  a  lowered  resistance  to  infection. 
In  certain  instances  where  nursing  is  exceptionally  good 
and  where  no  such  infection  occurs,  the  patient  con- 
tinues to  grow  weaker  and  weaker,  paralyses  of  all  the 
muscles  follow  and  finally  paralysis  of  deglutition  or 


198  PUZZLES   AND    ERRORS 

respiration  may  lead  to  death.  The  emaciation  and 
paralyses  may  be  of  such  a  grade  that  the  patient  is 
entirely  devoid  of  fat  and  unable  to  move  at  all.  Not 
infrequently  vascular  crises  occur,  and  one  of  these  may 
be  responsible  for  death. 

2.  What  was  the  cause  of  the  hemitremor?  The  hemi- 
tremor  suggested  an  irritative  or  destructive  lesion  in 
the  motor  path.  Delving  into  the  history  it  was 
learned  that  the  patient  had  had  a  shock  followed  by 
a  right  hemiparesis.  This  had  cleared  up  leaving  the 
tremor  as  a  residuum.  The  autopsy  disclosed  a  reddish- 
brown  pigmentation  and  fibrous  thickening  of  the  pia 
over  the  left  motor  area,  confirming  the  idea  of  a 
previous  hemorrhage.  As  a  rule  the  shock  phenomena 
occurring  in  paresis  clear  up  more  completely  and  no 
gross  lesion  is  visible  post-mortem.  However,  cerebral 
hemorrhage  must  be  expected  in  any  person  suffering 
from  syphilis,  and  is  no  rarity  in  paretic  neurosyphilis. 


PUZZLES  AND   ERRORS  199 


PARETIC  NEUROSYPHILIS  ("  general  paresis  ") 
with  NORMALLY  REACTING  PUPILS.  History 
of  trauma.  Autopsy. 


Case  51.  Daniel  Wheelwright,  a  barber  of  English  ex- 
traction, 57  years  of  age,  had  had  a  sunstroke  at  15.  At  42, 
there  had  been  pneumonia,  after  which  an  attack  of  rheuma- 
tism was  said  to  have  kept  the  patient  from  work  for  a  year. 
There  was  trauma  of  head  (falling  wrench)  at  44.  This  blow 
on  the  head  was  the  assigned  cause  of  the  mental  disease, 
symptoms  of  which,  however,  did  not  develop  until  about  the 
first  of  September,  1905,  about  three  months  before  entrance, 
January  9,  1906,  and  about  six  months  before  death,  March 
20,  1906. 

It  seems  that  the  patient  had  begun  to  change  in  manner; 
he  had  become  despondent  and  apathetic,  silent,  and  som- 
nolent. Two  weeks  later,  he  stopped  working,  began  to 
read  the  papers  once  more,  and  became  somewhat  more 
cheerful. 

About  Thanksgiving,  Wheelwright  got  up  at  midnight,  and 
remained  up,  lighting  all  the  fires  and  talking  continuously. 
During  the  next  two  weeks,  he  talked  much  to  himself, 
laughing  out  at  times.  About  two  weeks  before  Christmas 
he  went  out  and  started  to  make  a  sidewalk  of  old  boards, 
working  in  his  shirtsleeves,  without  a  hat.  He  would  work 
until  midnight  making  screens  for  windows.  During  the 
day,  he  would  go  out  and  give  money  to  passing  children; 
would  offer  to  pay  the  grocer  twice  as  much  as  articles  were 
worth. 

On  the  day  before  Christmas,  he  put  out  all  the  fires  and 
lights  in  the  house,  sent  all  the  family  to  bed,  and  opened  all  the 
doors.  Christmas  morning,  he  rose  early  and  got  the  washtubs 
ready.  He  helped  his  compliant  wife  to  do  the  washing,  then 
put  out  all  the  fires  and  opened  the  windows.  After  Christmas, 
he  began  to  tell  how  rich  he  was  going  to  be  through  starting 
a  garden  and  by  making  butter.  He  bought  six  or  seven 


2OO  PUZZLES  AND  ERRORS 

quarts  of  milk  daily,  and  procured  carrots  and  oranges, 
grinding  them  up  to  color  the  milk.  January  9th  he  was 
committed  to  Danvers  Hospital. 

Physically,  there  were  few  symptoms.  Neurologically,  there 
was  a  tremor  of  tongue,  fingers,  and  face.  The  knee-jerks 
were  lively.  The  pupils  reacted  normally;  the  patient  was 
restless,  pacing  up  and  down.  There  was  a  speech  defect 
demonstrable  with  test  phrases.  Orientation  was  imperfect 
for  time  and  for  place.  Hand-writing  was  poor,  memory 
impairment  was  marked,  but  the  patient  was  given  to  fabri- 
cation as  to  past  events.  A  characteristic  sample  of  state- 
ments: 

"  Do  you  know  that  this  is  an  insane  hospital?"  '  Yes; 
there  are  two  or  three  men  here  out  of  their  heads.  I  could 
cure  them  with  my  hands  but  they  won't  let  me.  I  could  get 
all  the  sick  men  on  their  feet  just  by  rubbing  them.  I  can 
do  anything  with  my  hands.  I  can  build  a  house  by  just 
sitting  down  and  thinking  about  it.  I  can  whip  all  the  men 
in  this  place.  I  have  better  sense  now  than  I  ever  had  in 
my  life." 

Again,  "  How  long  have  you  been  here?"  "  Over  three 
months;  they  have  put  me  in  heaven  three  times  since  I  have 
been  here.  They  killed  me,  crushed  my  heart,  and  turned 
my  blood  to  water.  I  am  all  right  now.  I  let  the  sun  shine 
on  my  heart  and  it  brought  it  together.  I  can  whip  every 
man  in  here  as  fast  as  they  come  up." 

Again,  "  I  will  make  a  million  dollars  on  my  garden  when 
I  get  it.  I  can  make  a  million  dollars  on  half  an  acre. 
I  can  do  anything.  I  can  move  this  house  by  just  thinking 
of  it." 

During  a  special  examination,  the  patient  told  how  he  had 
fastened  wings  on  his  hands  and  feet,  and  how  he  had  gone  to 
heaven;  he  told  how  he  had  soared  high  above  the  earth,  and 
how  differently  the  stars  look  when  up  near  heaven  than  they 
do  from  the  earth.  He  spoke  of  seeing  angels  and  of  the 
beauties  of  heaven. 

The  diagnosis  of  PARETIC  NEUROSYPHILIS  was  confirmed 
at  autopsy. 


PUZZLES   AND   ERRORS  2OI 

I.  What  is  the  significance  of  the  normally  reacting  pupils? 
While  it  is  usual  to  find  pupillary  anomalies  in 
neurosyphilis,  these  changes  are  not  an  essential  part 
and  it  is  not  rare  to  find  normal  pupils  in  all  forms  of 
neurosyphilis.  It  is  less  frequent  to  find  a  normal 
pupil  in  tabetic  than  in  diffuse  or  paretic  neurosyphilis. 
In  paretic  neurosyphilis  it  is  the  rule  to  find  pupillary 
changes  during  some  stage  of  the  disease,  but  not  neces- 
sarily early.  At  times  the  pupillary  sign  may  be  one  of 
the  earliest  signs  of  neurosyphilis  —  again  it  may  occur 
only  as  a  late  symptom,  if  at  all.  One  of  the  most  im- 
portant of  the  pupillary  signs  is  irregularity  of  contour. 
While  this  does  not  always  mean  neurosyphilis  it  is 
highly  suggestive  and  certainly  indicates  careful  exami- 
nation even  though  the  W.  R.  in  the  blood  be  negative. 

2  'What  was  the  relation  of  trauma  to  the  development 
of  the  neurosyphilitic  symptoms?  It  is,  of  course,  the 
rule  in  all  forms  of  mental  disease  to  have  some  factor 
offered  by  the  patient  or  relatives  as  the  cause  of  the 
psychosis.  Often  these  assigned  causes  are  minor 
events  thought  of  only  after  the  later  appearance  of 
symptoms.  In  this  case  it  was  not  thought  that  the 
trauma  had  any  causal  effect.  For  a  discussion  of 
trauma  and  neurosyphilis  see  cases  Joseph  O'Hearn 
(90),  Levi  Sussman  (91),  and  Joseph  Larkin  (92). 


2Q2  PUZZLES   AND   ERRORS 


NEUROSYPHILIS,  probably  PARETIC,  with 
symptoms  highly  suggestive  of  MANIC-DEPRES- 
SIVE PSYCHOSIS. 


Case  52.  Bessie  Vogel  *  was  admitted  to  the  Psycho- 
pathic hospital  New  Year's  day,  1915,  in  a  very  much  ex- 
cited condition.  The  family  history  is  very  meagre,  and  all 
that  is  of  significance  is  that  mother  has  always  been  very 
"  nervous."  The  records  in  part: 

Past  History.  Very  healthy  as  a  child,  and  except  for  oc- 
casional throat  trouble  and  headache  had  no  physical  ailments 
until  eight  years  ago,  when  she  had  an  operation  for  appen- 
dicitis, and  two  and  one-half  years  ago  was  operated  upon  for 
hernia  and  adhesions.  Following  this  she  began  to  show  a 
lack  of  energy,  neglected  her  housework,  was  much  depressed, 
wept  frequently,  complained  constantly  of  pain  in  various 
places,  and  was  ill-tempered.  In  about  five  months  she 
improved,  and  then  after  a  couple  of  weeks  at  the  shore 
seemed  entirely  well. 

Present  Illness.  In  November,  1914,  that  is,  about 
seventeen  months  after  the  recovery  from  the  previous  de- 
pression, she  again  began  to  show  practically  the  same 
symptoms.  She  was  depressed,  could  not  sleep,  and  would 
get  up  in  the  night  and  sew;  was  self-centered  and  hyper- 
sensitive, then  became  restless  and  nervous;  wanted  to  go 
shopping  and  out  for  dinner;  went  to  New  York  and  then  to 
New  Bedford.  Symptoms  became  more  marked;  she  be- 
came very  ill-tempered,  threatened  her  husband  when  angry 
over  trifles,  threatened  suicide,  then  began  to  get  active  and 
spent  money  extravagantly.  At  the  end  of  two  months,  that 
is,  Jan.  I,  1915,  she  was  admitted  to  the  hospital. 

Physical  Examination.  A  small,  thin  woman,  appearing 
to  be  about  45  years  old  (actual  age  37).  Aside  from  the 

*  Reprinted  from  an  article  by  Southard  &  Solomon: 
"  Latent  neurosyphilis  and  the  Question  of  Paresis  sine 
paresi."  Boston  Medical  and  Surgical  Journal,  XXIV,  i. 


PUZZLES   AND   ERRORS  2O3 

absence  of  teeth  and  the  operation  scars,  the  general  exam- 
ination is  negative.  Neuromuscular  system:  The  pupils 
are  round,  regular,  equal,  and  react  to  light  and  accom- 
modation, but  do  not  hold  very  well.  Extraocular  move- 
ments well  performed,  no  palsies  of  facial  muscles,  tongue 
protruded  medially  without  tremor.  Uvula  is  raised  sym- 
metrically. Biceps  and  triceps  and  supinator  reflexes  are 
present  and  brisk.  Patellar  and  Achilles  reflexes  are  equal 
on  the  two  sides  and  brisk.  Abdominal  skin  reflexes  not 
obtained.  Plantar  reflex  active  and  flexor  in  type.  No 
Babinski,  Gordon,  or  Oppenheim.  No  tremors. 

Wassermann  reaction  serum  positive.  Examination  of 
spinal  fluid:  clear,  globulin  +  +  +  +,  albumin  +  +  +  +  ; 
cells,  130  per  cmm.;  small  lymphocytes,  79.9%;  large  lym- 
phocytes, 14.1%;  polymorphonuclear  leucocytes,  4.6%; 
plasma  cells,  0.7%;  endothelial  cell,  0.7%.  W.  R.  positive. 
Gold  sol  reaction,  55555S22  +  -• 

Mental  Examination.  On  admission  patient  showed  great 
psychomotor  activity,  was  very  playful,  marked  flight  of 
ideas,  was  expansive,  very  emotional,  very  erotic.  She 
slept  very  little,  appetite  was  poor,  and  she  lost  weight 
rapidly.  Orientation  and  memory  intact.  No  hallucinations 
elicited.  In  about  three  weeks  improvement  began,  and  at 
the  end  of  eight  weeks  she  appeared  practically  recovered. 
On  April  9,  1915,  —  that  is,  13  weeks  after  admission,  — 
she  was  allowed  home  on  visit.  On  leaving,  she  appeared 
normal  in  every  way.  There  was  no  evidence  of  psychotic 
symptoms,  she  had  good  insight,  and  physically  there  was 
absolutely  nothing  of  a  neurological  nature  that  was  abnormal. 
"This  case,  with  the  history  of  a  previous  depression  and  its 
clinical  picture  during  the  acute  stage,  and  its  recovery, 
is  certainly  in  every  respect  typical  of  manic-depressive  in- 
sanity, and  only  the  positive  result  of  the  six  tests  causes  us 
to  put  it  in  the  group  of  GENERAL  PARESIS.  Only  the  further 
course  will  shed  any  light  as  to  the  correct  significance  of 
these  findings,  and  even  then  we  shall  not  be  too  sure  that  we 
had  not  been  dealing  with  a  manic-depressive  psychosis  in  a 
latent  neurosyphilitic.  We  would  strongly  emphasize  the 
point  that  at  the  present  time  this  patient  presents  no  mental 


204  PUZZLES  AND  ERRORS ' 

or  physical  signs  of  cerebrospinal  syphilis  or  general  paresis; 
but  the  six  tests  are  still  positive.  This  case  differs  from  the 
ordinary  general  paresis  remission  in  that  there  is  not  a  single 
physical  sign  of  paresis  present. 

There  are  many  transitional  cases  between  this  case  which 
shows  no  symptoms  or  signs  of  neurosyphilis  except  the  lab- 
oratory tests,  and  the  typical  case  of  general  paresis.  Thus 
we  have  cases  with  slight  character  change  and  no  physical 
signs  except  rare  "  seizures."  On  the  other  hand,  in  many 
cases  the  presence  of  abnormal  neurological  phenomena  with- 
out definite  mental  signs  is  first  noted.  Certain  remitted 
cases  show  only  some  slight  pupillary  or  reflex  abnormality. 
We  believe"we  have  here  added  the  last  link  in  the  chain 
between  the  primary  and  quaternary  symptoms. 

This  case  is  illustrative  of  several  which  we  have  pub- 
lished elsewhere  under  the  name  of  paresis  sine  paresi  or 
latent  neurosyphilis  to  illustrate  how  all  the  laboratory  signs 
of  neurosyphilis  may  be  present  in  a  patient  without  any 
physical  or  mental  symptoms  that  may  be  correlated  with 
these  findings. 

We  summarize  our  discussion  of  this  as  follows: 

1.  There  is  a  group  of  cases  showing  the  laboratory  signs 
characteristic  of  central  nervous  system  syphilis :     (a)  positive 
W.  R.  in  the  serum,  (b)  positive  W.  R.  in  the  spinal  fluid, 
(c)  pleocytosis,  (d)  excess  of  albumin,  and  (e)  of  globulin  in 
the  spinal  fluid,    (/)   gold  sol  reaction  of  central  nervous 
system  syphilis,  and  which  show  no  sign  or  symptom  of 
neurosyphilis. 

2.  We  believe  these  cases  represent  a  form  of  chronic 
cerebrospinal  syphilis,  probably  paretic  in  type. 

3.  They    have    the    greatest    theoretical    and    practical 
significance  in  the  consideration  of  the  life  history  of  neural 
syphilis,  in  the  concept  of  Allergie,  in  regard  to  results  of 
treatment,  and  finally  as  to  the  evaluation  of  the  laboratory 
tests. 

4.  Here  is  perhaps  offered  the  last  link  to  form  a  complete 
chain  between  the  symptoms  of  the  primary  stage  of  syphilis 
and  its  final  termination  of  life  as  the  result  of  the  diseases 
cerebrospinal  syphilis  or  general  paresis. 


PUZZLES  AND   ERRORS  2O5 


SYPHILIS  (?);  EXOPHTHALMIC  GOITRE;  neu- 
rosyphilitic  old  lesion  of  optic  thalamus ;  unilateral 
induration  and  atrophy  of  left  cerebral  cortex. 
Autopsy. 


Case  53.  Carrie  Pearson,  a  housewife  25  years  of  age,  died 
at  Danvers  Hospital  less  than  a  week  after  admission,  and  it 
was  at  first  stated  that  her  symptoms  had  lasted  but  two 
weeks  before  admission.  In  point  of  fact,  a  further  investi- 
gation showed  an  important  succession  of  symptoms,  lasting 
some  four  years. 

Carrie  had  been  considered  a  healthy  child,  going  to  school 
at  the  usual  age,  and  progressing  well  with  her  studies.  She 
however,  left  school  in  the  ninth  grammar  grade,  at  the  age 
of  15,  and  went  to  work  in  a  milltown.  She  married  a  worth- 
less person  at  the  age  of  18,  and  lived  with  her  husband 
for  three  years.  There  was  one  child  born  a  year  after 
marriage.  Two  years  later,  however,  a  tremendous  goitre 
had  developed  such  that  her  neck  was  described  as  "  out 
square  with  the  face,"  and  at  the  same  time  the  patient's 
eyes  had  become  prominent. 

About  two  weeks  before  admission,  she  had  gone  to  a 
neighboring  town  to  take  care  of  a  sick  woman,  but  during 
her  endeavor  to  be  a  nurse,  she  had  broken  out  into  a  mania, 
tearing  up  furniture  and  bedding,  and  talking  irrelevantly 
for  a  period  of  four  days.  She  also  showed  insomnia  and 
continually  tore  off  her  clothing  from  her  body. 

Upon  examination,  the  marked  enlargement  of  the  thyroid 
gland  together  with  the  prominent  eyeballs,  husky  voice,  and 
pulse  rate  of  150  per  minute,  were  entirely  consistent  with 
the  diagnosis  of  exophthalmic  goitre.  The  patient  described 
herself  as  "  Carrie  Nation."  Asked  to  write  her  name, 
she  took  the  pen  and  tried  to  spatter  ink,  wrote  hurriedly  and 
carelessly  her  maiden  name  and  several  words  without  ap- 
parent meaning.  Asked  to  write,  "  God  save  the  Common- 
wealth of  Massachusetts,"  she  wrote:  "  God  save  the 


2o6  PUZZLES   AND   ERRORS 

common  pal  U  S  Spe  Manor  Gen,  or  til  pat.  Since  Lord,  or 
no  prime  in  Hear  to  the  God  Tel.  Ho.  n  and  or  Mabel,  or  gal." 
After  this,  she  took  paper  and  wrote  meaningless  scrawls, 
saying  that  it  was  Japanese  writing.  There  was  much 
motor  restlessness  with  distractibility,  pointing  and  gri- 
macing, mimicking  the  actions  of  those  about  her. 

Death  occurred  from  exhaustion,  and  the  case  might  not 
have  been  regarded  as  unusual  except  for  the  autopsy,  which 
showed  a  peculiar  brain  lesion,  described  below.  The  point 
of  greatest  interest  in  the  case  was  the  fact  that  syphilis  is, 
although  not  proved  to  exist  by  laboratory  tests,  beyond 
question  a  factor  in  the  case.  Although  the  woman  had 
given  birth  to  a  normal  child,  who  is  still  alive,  yet  in  the 
period  of  a  few  years  her  breasts  had  atrophied,  her  hair  had 
disappeared  from  the  axilla  and  from  the  pubes;  varicose 
veins  had  developed  in  both  legs.  Whereas  there  was  little 
or  no  fat  over  the  chest  or  back,  the  omentum  and  mesentery 
were  very  plentifully  supplied  with  fat.  It  is  probable,  then, 
that  we  are  dealing  with  a  case  of  exophthalmic  goitre  some- 
how of  syphilitic  origin.  The  brain  lesion  is  consistent  with 
this  hypothesis. 

Autopsy,  March  3,  1907.     Four  hours  post-mortem. 

Body  length,  165  cm.  Body  of  a  well  developed  and 
well  nourished  young  woman.  Lividity  in  dependent 
parts.  Purplish  discoloration  of  left  thigh  to  knees. 
Skin  rough  and  scaly.  Petechial  eruption  over  chest. 
Neck  thick,  protrudes  anteriorly.  Varicose  veins  over 
upper  parts  of  calves  on  both  legs.  Eyes  protruding, 
not  covered  entirely  by  lids.  Pupils  equal,  dilated. 
Subcutaneous  fat  very  deep  over  lower  part  of  body. 
Very  little  fat  over  chest  and  back.  Breasts  are  very 
small,  apparently  atrophied.  Normal  amount  of  hair 
on  head,  slight  amount  over  pubes.  Axillary  hair  ab- 
sent. Fat  on  section  of  a  light  yellow  color.  Omentum 
extends  to  pubes,  plentifully  supplied  with  fat.  Large 
amount  of  mesenteric  fat.  Appendix  normal.  In- 
testines smooth  and  glistening.  Slightly  injected.  No 
fluid  in  peritoneum.  Uterus  small,  retroverted. 

HEAD:  HAIR  in  good  quantity.  SCALP  normal. 
CALVARIUM  shows  diploe.  DURA  MATER  over  left 
cerebral  hemisphere  inseparably  adherent  to  calvarium, 


iv>.;  *H  •;  •<»•••  s.vr-vv:r 
-  »   *••   i  >••';*,   *  H^    /..{•  ,. 
*...'.  •  t"  ^ ••*,;•,  ?:» ;>. 


--..,  -  v  -%J     '     '  • 

»',       v".  .         •   >     v  '<    '••         '  • 

:  v.-:-—--,''   "  ,-    >; 

.    '. 
4  x- --;:.;\ :•%-  ,  •    >• 


Cortical  hemiatrophy  —  A,  relatively  normal  right  precentral  (  "motor"  )  cortex;     B, 
atrophic  left  precentral. 
Note  in  B: 

1.  Absence  of  giant  pyramids  of  Betz  (corticospinal,  upper  motor  neurones). 

2.  Superficial  (subpial)  condensation  of  tissues  with  sclerosis  (gliosis).      The  tissues 
in  all  areas  examined  on  the  left  side  yielded  this  effect. 


PUZZLES   AND   ERRORS  2O/ 

over  right  hemisphere  normal.  Arachnoidal  VILLI 
moderately  developed.  PIA  MATER  shows  injected 
veins,  notably  in  the  sulci  of  the  right  hemisphere. 
Pia  mater  everywhere  thin  and  clear.  VESSELS  at  base 
of  normal  appearance. 

^  BRAIN  weight  1180  grams.  Spread  on  a  board,  the 
right  hemisphere  tends  to  flatten  so  that  it  measures 
1.5  cm.  more  from  side  to  side  than  its  fellow.  Be- 
sides more  marked  venous  injection,  the  right  hemi- 
sphere shows  also  flatter  and  slightly  more  plastic 
convolutions.  The  posterior  poles  of  the  hemispheres 
are  a  little  firmer  than  the  parts  anterior.  The  orbital 
and  hippocampal  gyri  on  the  right  side  are  a  little 
firmer  than  the  surrounding  parts.  On  section  the 
gray  and  white  matter  shows  no  lesions,  excepting  the 
slight  plasticity  of  the  tissues  at  large  on  the  right  side 
and  a  well  marked  induration,  with  retraction  under 
the  knife,  of  the  occipital  and  hippocampal  white 
matter.  The  basal  ganglia  of  the  left  side  are 
normal.  On  the  right  side  a  sagittal  section  demon- 
strates a  rounded  area  of  induration,  with  ill-defined 
borders,  measuring  perhaps  1.5  cm.  from  above  down- 
wards by  2  X  2  cm.,  situated  largely  in  the  lenticular 
nucleus  and  involving  the  greater  portion  of  the  globus 
pallidus,  a  small  segment  of  the  putamen  below  and 
behind  and  the  regionary  part  of  the  anterior  com- 
missure with  surrounding  tissues.  The  most  striking 
feature  of  this  lesion  is  the  occurrence  in  the  middle 
of  a  cluster  of  vacuoles  or  cystic  clefts,  with  smooth 
pale  interiors,  ranging  from  pin-head  to  0.25  cm.  or  even 
0.5  cm.  in  greatest  diameters.  There  are  six  to  eight 
clefts  to  a  surface  of  section.  The  color  of  the  lesion 
differs  little  from  that  of  the  globus  pallidus  itself,  but 
the  tissue  is  a  trifle  translucent.  It  is  impossible  to 
demarcate  the  lesion  with  the  eye.  Induration  is  de- 
monstrable several  mm.  beyond  the  visible  part  of  the 
lesion.  The  consistence  of  the  lesion  slightly  surpasses 
the  usual  consistence  of  the  olivary  bodies. 

CERREBELLUM,  PONS  and  BULB  weight  165  grams. 
Cerebellar  tissue  a  trifle  more  plastic  than  usual. 
The  right  olive  is  not  so  prominent  as  usual. 

Note.  THYROID:  Weight  125  grams.  Both  lobes 
and  isthmus  enlarged.  One  lobe  more  than  the  other; 
lobe  on  one  side  measuring  6x4  cm. 


2o8  PUZZLES  AND   ERRORS 

Anatomical  Diagnoses 

Enlargement  of  thyroid  gland. 

Exophthalmos  with  dilated  pupils. 

Fatty  degeneration  of  thoracic  muscles. 

Slight  aortic  sclerosis. 

Dilatation  of  right  heart.  ^ 

Hypertrophy  of  left  ventricle. 

Slight  tricuspid  endocarditis. 

Bicuspid  aortic  valve. 

Hypostatic  pneumonia. 

Acute  and  chronic  splenitis. 

Fatty  liver  (central  necroses?). 

Acute  nephritis. 

Chronic  gastritis. 

Small  breasts. 

Axillary  hair  absent. 

Petechial  eruption  of  chest. 

Varicose  veins. 

Chronic  external  adhesive  pachymeningitis  of  left  side. 

Moderate  swelling  of  right  hemisphere  with  venous 
injection. 

Slight  occipital  gliosis  of  both  sides. 

Slight  gliosis  of  orbital  and  hippocampal  gyri  of 
right  side. 

Sclerosis  with  atrophy  of  occipital  and  hippocampal 
white  matter  of  right  side. 

Gliotic  lesion  (1.5  x  2  X  2  cm.  of  right  lenticular 
nucleus  involving  anterior  commissure). 

1.  Was  the  exophthalmic  goitre  in  Carrie  Pearson  due  to 

syphilis?  Unfortunately  we  have  no  clear  proof  that 
Carrie  Pearson  was  syphilitic.  She  was  stated  to  have 
been  syphilitic  by  the  physician  who  treated  her  before 
her  commitment  to  Danvers  Hospital.  There  is,  how- 
ever, no  proof  of  syphilis,  inasmuch  as  the  patient  died 
in  the  pre-Wassermann  period. 

2.  Is  the  thalamic  lesion  probably  syphilitic?     No  lympho- 

cytosis  or  plasmocytosis  characterizes  the  lesion,  which 
is  the  only  lesion  of  the  sort  in  the  Danvers  collection. 
It  would  not  do  to  call  a  lesion  syphilitic  just  because 
it  is  sui  generis.  In  any  event,  the  clinical  analysis  of 
the  case  faced  the  claim  of  syphilis  as  an  actual  factor 
in  the  patient's  life  and  as  a  possible  factor  in  the  goitre. 


PUZZLES   AND   ERRORS  2Og 


It  is  well  known  that  the  ARGYLL-ROBERTSON 
PUPIL  is  characteristic  of  the  so-called  "  PARA- 
SYPHILITIC  DISEASES"  ("  general  paresis" 
and  "  tabes  ") ;  does  this  sign  occur  in  other  neuro- 
syphilitic  conditions? 


Case  54.  Julius  Kantor  was  a  shoemaker  of  35  years, 
who  came  to  the  hospital  for  treatment  because  his  family 
physician  had  found  a  positive  W.  R.  in  Kantor's  blood 
serum.  He  had  had  a  cough  for  a  number  of  years,  and 
during  the  last  year  a  little  blood  had  been  found  in  the 
sputum;  whereupon  Kantor  had  been  placed  under  active 
anti-tuberculosis  treatment.  The  enterprising  family  physi- 
cian had  found  the  positive  W.  R.  in  the  first  days  of  his 
treatment  for  tuberculosis.  There  was,  in  fact,  a  history 
of  a  chancre  nine  years  before,  which  had  not  been  followed 
by  any  secondary  or  tertiary  symptoms,  and  which  had 
been  but  scantily  treated. 

There  were  no  mental  symptoms. 

Kantor  was  physically  fairly  well  developed  and  nourished. 
There  were  a  few  piping  relies  in  the  left  upper  chest,  both 
in  front  and  back,  and  also  a  slight  dulness  with  increased 
vocal  and  tactile  fremitus.  No  tubercle  bacilli,  however, 
could  be  found  on  repeated  sputum  examination. 

Neurologically,  the  pupils  were  myotic  and  both  showed 
the  Argyll- Robertson  reaction.  There  were  no  abnormal 
reflexes  whatever,  and  there  was  neither  ataxia  nor  speech 
defect.  Not  only  the  blood  but  also  the  spinal  fluid  W.  R. 
proved  to  be  positive;  there  was  a  marked  increase  in  the 
albumin  and  globulin;  there  was  a  gold  sol  reaction  of  the 
syphilitic  type,  and  there  were  but  three  cells  per  cmm. 

I.  In  view  of  the  headache  in  case  Kantor,  what  other 
causes  of  headache  are  to  be  considered?  It  is  cer- 
tain that  irritations  of  the  dura  mater  can  produce 
headache,  and  the  physiological  observation  of  the 
sensitiveness  of  the  membranes  and  the  non-sensitive- 


2IO  PUZZLES   AND   ERRORS 

ness  of  the  brain  substance  is  an  ancient  and  classical 
observation.  Internal  hemorrhagic  pachymeningitis 
produces  severe  headache.  The  relations  of  this  dis- 
ease to  trauma,  to  arteriosclerosis,  and  possibly  to 
syphilis  (alcohol  perhaps  should  also  be  considered)  in 
certain  instances  have  not  been  entirely  cleared  up. 
Syphilitic  headaches  are,  according  to  Lewandowski, 
dependent  also  upon  a  dural  affection  or  upon  a  perios- 
teal  affection.  The  headaches  of  brain  tumor  are  also 
commonly  related  to  dural  conditions,  either  directly 
due  to  the  pressure  of  the  tumor  itself,  or  indirectly  to 
the  heightened  intracranial  pressure  consequent  upon 
the  tumor.  It  is  clear  that  the  tension  under  which  the 
dura  mater  lies  is  not  always  localized  in  the  region 
of  a  brain  tumor  or  a  syphilitic  lesion.  Head  has 
claimed  that  brain  tumor  produces  headaches  of  two 
kinds,  according  to  whether  the  disease  affects  the  dura 
mater  or  is  dependent  upon  an  increase  of  pressure  in 
the  brain.  It  does  not  appear  that  the  pia  mater  has 
any  relation  to  headaches,  but  meningitis,  in  which 
the  inflammation  is  confined  to  the  pia  mater,  is  never- 
theless associated  with  headache;  the  headache  is  here 
supposed  to  be  due  to  the  increase  in  brain  pressure, 
and  thus  actually  to  an  effect  wrought  upon  the  dura 
mater.  Vasomotor  disorders  and  various  types  of 
cephalic  hyperemia  are  thought  to  produce  a  kind  of 
headache,  but  Lewandowski  calls  this  kind  of  head- 
ache somewhat  in  question.  Reflex  headaches  are 
stated  to  be  produced  indirectly  by  a  process  of  radi- 
ation from  interior  lesions  in  the  brain.  There  are 
certain  headaches  called  nodal  headaches  (Schwielen- 
Kopfschmerz) .  Hypermetropia,  caries  of  the  teeth, 
adenoids,  and  diseases  of  the  nose  and  axillary  cav- 
ities, to  say  nothing  of  thoracic  and  abdominal  diseases, 
are  also  counted  among  conditions  that  may  produce 
headaches.  In  this  connection,  Head  has  claimed 
differential  zones  of  headache  corresponding  to  certain 
diseases. 

The  brain  itself  may  produce  headache  through  in- 
toxications, through  conditions  produced  by  a  variety 
of  diseases;  may  follow  neuroses.  Alcohol  may  pro- 
duce headaches  in  some  persons  even  when  it  is  taken 
in  very  small  doses.  Certain  uremic  cases  yield  head- 
aches, as  do  also  gouty  and  chlorotic  conditions. 
According  to  Lewandowski,  the  headaches  of  arterio- 
sclerotics  are  due  possibly  to  vasomotor  disturbances 


PUZZLES  AND   ERRORS  211 

in  the  membranes,  or  one  may  think  of  nutritive  cere- 
bral disorders.  A  peculiar  form  of  headache  is  that  of 
fatigue  after  mental  work,^allied  to  which  is  the  neuras- 
thenic headache;  constitutional  headaches  have  been 
assumed  to  occur,  to  say  nothing  of  hysterical  head- 
aches. There  remains  also  the  important  question  of 
migraine,  for  which  a  vasomotor  explanation  has  been 
proposed. 

2.  Was  Kantor  suffering  from  tuberculosis  of  the  lungs? 

The  hypothesis  of  lung  syphilis  ought  certainly  to  be 
very  seriously  considered.  Upon  repeated  sputum 
examination,  no  tubercle  bacilli  have  yet  been  found. 

3.  Is  Kantor  a  case  of  general  paresis?     In  the  absence  of 

mental  symptoms,  and  in  consideration  of  the  mild- 
ness of  the  reactions,  it  is  certainly  not  easy  to  make 
the  diagnosis  of  general  paresis.  However,  the  diag- 
nosis of  tabes  dorsalis  is  not  justified  either.  Accord- 
ingly, we  may  answer  our  question:  whether  the 
Argyll- Robertson  pupil  occurs  in  other  neurosyphilitic 
diseases,  by  pointing  out  that  in  the  case  of  Julius 
Kantor,  as  in  the  case  of  Henri  Lepere  (105)  and 
Frederick  Stone  (106),  the  Argyll-Robertson  pupil  has 
been  found  in  syphilitic  conditions  that  are  neither 
typically  paretic  nor  typically  tabetic. 


212  PUZZLES  AND  ERRORS 


Does  the  Argyll-Robertson  pupil  necessarily  indi- 
cate neurosyphilis? 


Case  55.  Daniel  Falvey,  44  years  of  age,  was  an  alms- 
house  transfer  to  the  Danvers  State  Hospital  in  the  year  1904, 
when  the  principle  of  state  care  was  adopted  in  Massachu- 
setts. As  in  most  of  the  almshouse  transfers  of  that  day, 
little  could  be  discovered  as  to  antecedents.  He  had  been  a 
mill-worker  from  the  time  of  his  immigration  in  1890,  at  30 
years  of  age.  He  had  been  somewhat  alcoholic.  There  was 
a  shock  some  17  months  before  his  death,  which  occurred 
about  seven  weeks  from  the  date  of  transfer. 

Not  only  was  he  unable  to  walk  unsupported,  but  when 
supported  there  was  a  slight  dragging  of  the  left  leg  and  the 
gait  was  noted  to  be  somewhat  propulsive.  The  tongue  and 
hands  were  tremulous,  and  the  left  grasp  was  somewhat 
weaker  than  the  right.  Both  knee-jerks  were  increased 
although  neither  more  than  the  other.  There  was  no  sensory 
disorder. 

Although  but  44  years  of  age,  Falvey  presented  the  appear- 
ance of  a  much  older  man.  His  heart  was  somewhat  enlarged 
and  there  was  a  degree  of  peripheral  arteriosclerosis.  On  the 
whole,  no  special  attention  was  attracted  to  this  case  clin- 
ically and  he  was  regarded  as  an  example  of  arteriosclerotic 
dementia,  like  many  another  among  the  transfers.  However, 
we  owe  to  Dr.  H.  M.  Swift  the  important  observation  of  the 
Argyll-Robertson  pupils.  The  case  was  studied  long  before 
the  Wassermann  method  was  available,  and  is  here  reported 
merely  to  call  attention  to  the  fact  that  the  stiff  pupils  may 
have  other  neural  origin  than  neurosyphilis. 

The  autopsy  material  in  the  case  was  worked  up  by  one  of 
the  authors.*  The  autopsy  had  been  performed  by  Dr.  A.  M. 
Barrett,  who  found  on  section  through  the  brain  stem  at  the 

*  E.  E.  Southard.  A  case  of  glioma  of  the  pineal  region, 
Am.  Jour,  of  Ins.,  Vol.  LXI,  1905. 


PUZZLES   AND   ERRORS  213 

anterior  border  of  the  pons  a  mass  springing  from  and  contin- 
uous with  the  pineal  gland,  lying  in  the  third  ventricle  and  the 
aqueduct  of  Sylvius.  Upon  further  study,  this  mass  was 
found  to  begin  posteriorly  in  the  pineal  body  itself,  from 
which  the  mass  could  hardly  be  told  in  the  gross  except  by  an 
injected  border. 

This  mass  proved  upon  microscopic  examination  to  be  a 
psammoma,  which  histologically  resembled  a  glioma  rather 
than  a  sarcoma.  Throughout  the  mass  there  was  a  variable 
content  of  fibrillary  intercellular  substance  having  the  histo- 
logical  reactions  of  neuroglia  fibrillae.  The  histological 
details  (mitosis,  large  giant  cells  with  multiple  nuclei,  etc.)  do 
not  here  concern  us.  We  deal  with  a  neoplasm  springing  from 
the  pineal  gland  growing  on  the  posterior  half  of  the  third 
ventricle,  the  anterior  orifice  of  the  aqueduct  of  Sylvius,  and 
the  space  between  the  velum  interpositum  as  far  back  as  the 
posterior  corpora  quadrigemina.  There  is  no  evidence  in  the 
body  of  old  syphilis;  although  it  is  possible  that  the  stiff 
pupils  were  neurosyphilitic,  it  seems  probable  that  they  were 
related  to  the  pineal  tumor.  At  all  events,  there  are  in  the 
literature  evidences  that  the  pineal-quadrigeminal  group  of 
tumors  and  other  lesions  may  bring  about  pupillary  distur- 
bances. On  this  account,  we  here  include  the  case.  The 
tumor  hardly  led  to  an  error  in  diagnosis  since  neither  neu- 
rosyphilis  nor  brain  tumor  was  at  all  expected  clinically. 

1.  Can   alcoholism    produce   identical    results?     See    Case 

Murphy,  (60),  one  of  alcoholic  pseudoparesis. 

2.  What  is  the  nature  of  stiff  pupils?     A  pupil  is  called 

stiff  in  the  sense  of  the  Argyll- Robertson  pupil  if  it 
fails  to  react  to  illumination  either  of  itself  or  of  the 
other  eye  and  at  the  same  time  if  it  reacts  properly  in 
convergence  and  accommodation.  Of  course  the  stiff- 
ness of  a  blind  eye  must  not  be  regarded  as  an  Argyll- 
Robertson  pupil.  In  a  case  of  right-sided  Argyll- 
Robertson  pupil,  therefore,  the  left  pupil  reacts  properly 
both  to  direct  illumination  of  itself  and  to  illumination 
of  the  right  eye,  but  the  right  eye  fails  to  react  to  illumi- 
nation of  either  eye.  Such  an  Argyll- Robertson  right 
pupil  will  remain  of  the  same  width  both  in  darkness 
and  in  light.  Clinicians  agree  that  the  Argyll- Robert- 


214  PUZZLES   AND   ERRORS 

son  is  diagnosticated  rather  too  frequently  than  too 
seldom,  and  this  by  reason  of  the  fact  that  a  sluggish- 
ness of  light  reaction  is  interpreted  as  stiffness.  The 
sign,  as  is  well  known,  has  come  to  be  regarded  as  almost 
pathognomonic  of  tabetic  or  paretic  neurosyphilis. 
Nonne,  however,  has  found  among  510  cases  of  alco- 
holism, nine  instances  of  Argyll-Robertson  pupil  and 
19  cases  of  sluggish  light  reactions.  The  pathological 
anatomy  of  this  sign  is  still  doubtful  although  a  number 
of  schematic  accounts  are  available;  among  hypotheses, 
one  may  think  of  an  elective  effect  of  the  tabetic  or 
paretic  degeneration  upon  reflex  collaterals.  The  ex- 
planation would  then  resemble  that  for  absent  knee- 
jerks  and  kindred  reflex  disorders.  We  should  then 
hypothesize  a  loss  of  the  finer  processes  of  the  terminal 
aborizations  about  the  cells  of  the  nucleus  of  sphincter 
nucleus  iridis.  However,  the  situation  of  the  sphincter 
iridis  has  not  yet  been  absolutely  determined. 

When  a  pupil  is  said  to  be  entirely  stiff  it  means  that 
it  reacts  neither  to  light  nor  accommodation.  This 
condition  not  infrequently  follows  the  partial  stiffness 
or  Argyll- Robertson  reaction. 

3.  Is  the  Argyll-Robertson  pupil  more  tabetic  than  paretic? 
This  has  been  claimed  at  times,  but  in  point  of  fact,  the 
Argyll-Robertson  pupil  is  very  frequent  in  paresis,  and 
so  also  are  posterior  column  changes.  According  to 
statistics  of  Bumke,  36%  of  tabetics  fail  to  show  the 
Argyll-Robertson  pupil,  and  38%  of  paretics.  When, 
however,  finer  methods,  such  as  those  standardized  by 
Weiler,  with  photographic  records,  are  employed,  the 
number  of  cases  without  at  least  a  tendency  to  the 
Argyll-Robertson  pupil  becomes  much  smaller. 

In  connection  with  the  important  question  as  to  the 
classical  Argyll-Robertson  pupil  and  pupillary  slug- 
gishness to  light,  it  may  be  inquired  what  are  the  ocular 
signs  in  neurosyphilis?  Joffroy  has  tabulated  the  signs 
in  300  general  paretics  as  follows: 

Sign.  No.  of  cases.  Per  cent. 

Alterations  of  light  reflex 235  78 

Inequality 205  68 

Abolition  of  light  reflex 156  52 

(bilateral  or  unilateral) 

Abolition  of  light  reflex 133  44 

(bilateral) 

Irregularity  of  pupil 117  39 


PUZZLES  AND   ERRORS  215 

Sign.  No.  of  cases.  Per  cent. 

Irregularity  of  both  pupils 109  36 

Diminution  of  light  reflex 108  36 

ditto  (bilateral) 79  26 

Alteration  in  accommodation  reflex.  79  26 

Diminution  of  accommodation  reflex  52  17 

Mydriasis 41  13 

Myosis 40  13 

Diminution  of  light  reflex 35  1 1 

(unilateral) 

Abolition  of  accommodation  reflex. . .  35  1 1 

Diminution  of  accommodation  reflex  29            9 

(bilateral) 

Abolition  of  accommodation  reflex  26             8 

(bilateral) 

Diminution  of  accommodation  reflex  23             7 

(unilateral) 

Fundus  changes 21             7 

Vascular  changes 16            5 

Abolition  of  accommodation  reflex. . .  12             4 

(unilateral) 

Paresis  of  the  third  nerves 10            3 

Ptosis 9             3 

Irregularity  of  one  pupil 8             3 

Nystagmus 7             2 

Visual  acuity  lost   7             2 

Atrophy  of  disc 6             2 

Total  blindness 5             2 

Paralysis  of  the  fourth  nerves I             I 


2l6  PUZZLES   AND   ERRORS 


Can  neurosyphilis  exist  in  the  absence  of  positive 
findings  in  the  spinal  fluid? 


Case  56.  There  was  no  great  difficulty  in  setting  up  a 
diagnosis  of  general  paresis  in  the  case  of  James  Burns,  a 
mechanic  of  31  years  of  age,  who  came  voluntarily  to  the 
Psychopathic  Hospital  for  treatment.  The  point  in  Burns' 
case  was  that  the  spinal  fluid  proved  entirely  negative  in  all 
respects  despite  the  fact  that  the  serum  W.  R.  was  positive, 
and  despite  the  following  facts  of  history  and  mental  exami- 
nation. 

The  patient  claimed  syphilitic  infection  seven  years  before, 
namely,  at  24  years  of  age,  and  also  claimed  that  he  had 
infected  his  wife,  who  was  in  fact  at  the  time  undergoing  anti- 
syphilitic  treatment.  He  complained  of  insomnia,  worry, 
depression,  hypersensitivity  to  noises  (such  as  those  made 
by  his  own  children),  thoughts  of  suicide,  and  amnesia.  The 
amnesia,  however,  might  be  regarded  as  subjective  since  our 
tests  failed  to  show  amnesia.  Nor  was  there  any  diminu- 
tion in  arithmetical  ability.  Despite  the  patient's  claim  that 
he  had  been  "  way  off  in  his  way  of  thinking,"  there  appeared 
to  be  no  delusions.  Beyond  a  certain  flightiness  in  conversa- 
tion, we  could  hardly  get  any  evidence  of  psychosis  unless  of 
the  neurasthenic  order. 

Physically,  however,  the  left  pupil  failed  to  react  to  light 
though  it  was  found  to  react  to  distance,  and  the  right  pupil 
exhibited  a  diminution  of  its  reaction  to  light.  There  was 
no  ataxia  of  gait,  yet  there  was  a  complete  Romberg  reaction. 
There  was  a  moderate  tremor  of  the  hands  and  of  the  tongue. 
Otherwise  there  were  no  reflex  disorders  upon  systematic 
examination,  nor  was  there  any  demonstrable  disorder  in  the 
rest  of  the  physical  examination. 


I.     What  is  the  diagnosis  in  the  case  of  James  Burns?  ^ 

the  whole  we  agree  with  Nonne,  that  negative  spinal 
fluid  findings  (of  course,  in  the  absence  of  treatment) 
preclude  the  diagnosis  of  general  paresis.  The  symp- 


PUZZLES  AND  ERRORS  217 

toms  might  possibly  be  explained,  however,  by  means 
of  a  localized  syphilitic  involvement  of  the  cerebrum, 
no  cells  or  products  of  inflammation  having  penetrated 
to  the  spinal  fluid.  According  to  Head  and  Fearnsides, 
this  condition  may  be  found  especially  in  the  anterior 
or  middle  fossa.  Accordingly,  going  upon  these  views 
of  Nonne  and  of  Head  and  Fearnsides,  we  should  be 
entitled  to  make,  perhaps,  a  diagnosis  of  cerebral 
syphilis. 

2.  What  is  the  significance  of  the  Argyll- Robertson  pupil  in 
James  Burns?  Nonne  states  that  if  one  follows  cases 
with  Argyll- Robertson  pupil  over  a  sufficient  period 
of  years,  they  one  and  all  eventuate  in  active  symptoms 
of  cerebrospinal  syphilis  (not  necessarily  of  the  cortical 
type) ,  and  this  despite  the  fact  that  the  pupillary  change 
may  have  been  present  a  number  of  years  before  any 
other  symptom  had  developed. 


2iS  PUZZLES   AND   ERRORS 


Neurosyphilis  ("  DISSEMINATED  ENCEPHA- 
LITIS") within  seven  months  of  initial  infection. 
Autopsy. 


Case  57.  We  borrow  the  main  features  of  a  remarkable 
case  examined  at  the  Danvers  State  Hospital  clinically  by 
Dr.  H.  W.  Mitchell  and  reported  elaborately  by  Dr.  A.  M. 
Barrett.  This  case,  whom  we  shall  call  John  Summers, 
acquired  syphilis  at  about  the  end  of  the  third  week  in  May, 
1902,  and  consulted  a  physician  on  June  12,  at  which  time 
a  characteristic  initial  lesion  of  syphilis  was  plain.  Summers 
was  excessively  alcoholic  at  times  and  was  not  seen  by  a 
physician  again  until  July  2,  just  after  an  alcoholic  debauch. 
At  this  time  there  was  ulceration  of  the  primary  lesion,  and  a 
papillary  eruption  had  developed  over  the  arms,  chest, 
abdomen,  and  legs.  Mercurial  treatment  and  mixed  treat- 
ment were  given.  Arthritis  occurred  but  disappeared  with 
increased  dosage. 

About  six  months  after  infection,  the  patient  developed 
severe  headaches,  hardly  controllable  by  treatment.  Amnesia 
and  a  certain  stupidity,  with  neglect  of  personal  habits,  and 
even  of  eating,  developed,  whereupon  Summers  was  admitted 
to  the  Danvers  Hospital,  December  n,  1902.  He  weighed 
124  pounds,  was  extremely  feeble,  with  dull  and  expression- 
less face,  coarse  purposeless  movements  of  arms;  left  pupil 
larger  than  right;  right  external  strabismus  and  ocular  ptosis; 
increased  knee-jerks,  crossed  adductor  reflex,  coarse  tremors 
of  arms  and  hands;  and  extreme  clouding  of  consciousness. 
It  was  doubtful  whether  the  pupils  were  stiff  to  light  or  not. 

The  patient  died  on  the  ninth  day,  December  18,  in  a 
state  of  coma.  After  admission,  his  stupor  had  become  more 
marked;  there  had  been  incontinence  of  urine  and  faeces, 
and  the  patient  could  be  aroused  only  by  loud  tones. 
Difficulty  in  swallowing  had  developed;  the  right-sided 
ptosis  had  become  more  marked,  and  muscular  twitchings 
had  developed  on  the  right  side.  When  the  left  leg  was 


I.    Exudate  in  pia  mater  —  mononucleosis. 


\  * 

-ft*' 

'*    *•     * 

v*       >•  ' 

» 


2.  Superficial  (subpial)  cellular 
reaction  of  neuroglia  tissue  (expand- 
ed cell  bodies). 


3.  Cellular  gliosis  of  deeper 
layers  of  cortex.  Apparent  in- 
crease in  capillary  supply,  possibly 
relative  to  loss  of  neural  elements. 


Case  57.     Neurosyphilis  ("disseminated  syphilitic  encephalitis"  of  A.  M.  Barrett),  fatal 
seven  months  from  initial  infection.     (Photographs  by  A.  M.  Barrett.) 


PUZZLES   AND   ERRORS  2IQ 

pinched,  there  was  twitching  of  the  left  leg  and  arm.  There 
was  slight  spasticity  of  the  right  arm  and  leg.  An  examination 
upon  the  day  of  death  definitely  showed  a  lack  of  reaction  of 
the  pupils  to  light. 

Dr.  Barrett  was  able  to  find  in  the  literature  a  case  of 
Bechterew  which  histologically  resembled  his  own  case,  but 
though  in  the  instance  reported  by  Bechterew  the  first  symp- 
toms developed  within  the  year  following  infection,  death  did 
not  occur  until  two  years  later. 

In  view  of  a  total  duration  of  symptoms  clearly  not  over 
seven  months,  it  is  interesting  to  inquire  how  far  micro- 
scopic brain  changes  could  have  proceeded.  Neither  cal- 
varium  nor  dura  mater  showed  changes.  There  was  a  slight 
haziness  of  the  pia  mater  over  the  convexity,  but  the  pia 
mater  over  the  base  (especially  below  the  cisterna  and  from 
thence  spreading  out  over  the  pons  and  into  the  fissure  of 
Sylvius)  was  not  only  hazy  but  definitely  thickened  and 
hyperaemic.  The  thickening  was  most  marked  about  the 
root  of  the  right  third  nerve  (corresponding  with  the  eye 
findings  in  life).  There  was  also  a  macroscopic  thickening  of 
the  left  Sylvian  artery.  Section  of  the  brain  showed  nothing 
abnormal  except  a  small  area  among  the  pyramidal  fibres 
of  the  right  side  of  the  pons,  where  there  was  a  single  hemor- 
rhagic  area  about  7  mm.  in  diameter  around  which  there 
were  small  punctiform  hemorrhages.  (Compare  twitchings 
of  left  leg  and  arm  upon  stimulation  of  left  leg,  and  note 
also  the  muscular  twitchings  and  slight  spasticity  of  right 
leg  and  arm  noted  just  before  death.)  This  case  was  exam- 
ined and  reported  upon  in  1905.  We  learn  from  Dr.  Barrett 
that  a  re-study  of  the  case  with  modern  methods  has  failed 
to  demonstrate  a  spirochetosis. 

The  meninges  show  infiltration  and  destructive  and  pro- 
liferative  changes  of  the  blood  vessels.  Condensed  extracts 
from  Dr.  Barrett's  full  report  follow: 

There  were  local  variations  in  the  severity  of  the 
meningitis.  The  sulci  showed  the  most  marked  in- 
filtration. The  slighter  degrees  of  exudation  were 
made  up  largely  of  lymphocytes  with  a  few  plasma 
cells,  occasionally  large  mononuclear  cells,  and  rarely 


22O  PUZZLES  AND  ERRORS 

a  polymorphonuclear  leukocyte.  Where  the  exudation 
was  more  extensive,  the  large  mononuclear  cells  became 
more  common  and  the  polymorphonuclear  leukocytes 
increased  in  number.  The  large  mononuclear  cells  were 
often  phagocytic,  containing  from  one  to  six  leukocytes. 
The  exudate  was  always  most  abundant  about  the 
blood  vessels.  The  plasma  cells  were  always  most 
numerous  in  the  adventitia  of  the  veins,  here  greatly 
outnumbering  the  leukocytes.  The  polymorphonuclear 
leukocytes  were  relatively  infrequent  except  where  there 
were  necrotic  areas,  which  areas  were  usually  con- 
tinuous with  an  infiltration  of  a  vessel  wall. 

As  to  vascular  changes,  the  media  was  not  often 
involved,  nor  was  the  adventitia  so  often  affected  as  the 
intima.  Such  lesions  as  appeared  in  the  intima  and 
adventitia  were  infiltrative  rather  than  proliferative. 
The  elastica  of  the  blood  vessels  proved  to  show  but 
slight  changes. 

A  characteristic  change  was  the  endarteritis,  —  of  a 
focal  nature  with  a  few  large  mononuclear  and  lympho- 
cytic  cells  pushing  the  intima  inward  at  the  edge  of  a 
lesion.  In  the  more  marked  portion  of  the  focal  process, 
the  thickness  of  the  intima  was  greatly  increased  by 
proliferation.  Great  numbers  of  large  mononuclear 
cells  could  be  seen  between  the  intima  and  the  elastica. 
About  these  cells  and  interlacing  among  the  other 
elements  of  the  proliferating  tissue  was  an  excess  of 
connective  tissue  fibres. 

The  meningeal  veins  were  more  often  diseased  than 
the  arteries;  there  was  adventitial  infiltration  with 
lymphoid  and  plasma  cells;  sometimes  the  vein  walls 
had  become  necrotic  and  infiltrated  with  polymorpho- 
nuclear leukocytes. 

It  will  be  remembered  that  the  left  Sylvian  artery 
was  grossly  thickened,  and  microscopic  section  of  this 
vessel  showed  a  partial  thrombosis. 

The  brain  showed  diffuse  and  focal  changes.  The 
diffuse  process  was  one  of  nerve  cell  degeneration  and 
proliferative  changes  in  the  neuroglia  and  blood  vessels, 
and  no  section  of  the  many  examined  proved  to  be  free 
from  such  changes,  although  in  the  majority  of  instances, 
these  diffuse  changes  were  slight.  The  cortical  layers 
showed  more  of  these  diffuse  changes  than  did  the 
white  substance.  Barrett  considered  that  the  glial  cell 
changes  were  more  delicate  indicators  of  the  cortical 
changes  than  the  nerve  cell  changes.  He  found  rod 


^Sw^Sp^jESKir 
%&^?>j£a&aw-S^ 

;'!^fe: 

'    *     -?&VV  ^ 


4.     Arteritis  of  pia  mater. 


*;Y  •-•••'•iy.^V  .-.#•••:.•     !,  '/'*:-C*$^*  ••*',•••  : 

'"'r       ' 


7V>if>>.'.l-'.xt    *>'  •  —    •     ••  •>•'     "-•r;"-. 

..  .-X  •..',••  -  •'    ^*.-  -•:  .. -^       :  -.'•.  v  '  •  •     -        • 

-.^•;^;--' "     -v  -.-•• 

-y-.<v;>^f.'  ;  •..  ' 


5.     Focal  vascular  lesions. 

Case  57.     Seven  months  from  infection.     "Disseminated  syphilitic  encephalitis,"  Barrett. 
(Photographs  by  Barrett.) 


Paretic  neurosyphilis  ("general  paresis")  —  cerebral  atrophy,  without 
meningitis.  Therapeutics  cannot  hope  to  restore  lost  tissue. 

Duration.  3  years  from  beginning  of  well  marked  symptoms;  6  years 
from  beginning  of  obvious  symptoms;  12  years  from  a  so-called  "  nervous 
prostration." 


PUZZLES  AND   ERRORS  221 

cells,  satellitosis,  superficial  gliosis,  and  a  large  gamut 
of  changes  in  the  neuroglia.  There  were  two  rather 
characteristic  nerve-cell  changes:  a  shrinkage  change 
going  on  to  almost  complete  destruction,  and  a  type  of 
cell  swelling,  also  apparently  proceeding  to  complete 
destruction. 

Among  focal  changes,  there  were  four  main  types: 
Areas  of  encephalitis,  having  the  general  appearance 
of  granulation  tissue,  areas  of  simple  necrosis  or  soften- 
ing, apparently  directly  related  to  vascular  changes 
near  by,  hemorrhages,  and  certain  foci  regarded  as 
gummatous. 

Save  for  pial  infiltration  and  a  few  vascular  changes, 
there  was  very  little  change  in  the  medulla  and  spinal 
cord.  There  was  a  hypertrophic  gliosis  of  the  margin 
of  the  medulla  and  cord  throughout,  and  a  focal  lesion 
of  well-marked  gliosis  at  one  point  in  the  bulb.  There 
were  no  fibre  degenerations  in  the  medulla  or  cord,  nor 
were  there  any  coarse  fibre  degenerations  in  the  cortex 
itself  except  in  one  locus,  the  left  gyrus  rectus.  This 
case  is  of  peculiar  value  in  showing  to  what  extent 
lesions  may  proceed  in  a  period  of  six  to  eight  months 
after  primary  infection. 

Of  course  the  case  is  in  one  sense  entirely  atypical.  The 
lesions  were  not  confined  to  the  nervous  system.  Aside  from 
the  maculo-papular  eruption  and  ecchymosis  of  the  skin, 
there  was  a  diffuse  hemorrhage  of  the  inner  half  of  the  con- 
junctiva of  the  left  eyeball,  a  small  hemorrhagic  focus  in  the 
mitral  valve,  a  caseous  nodule,  one  cm.  in  diameter,  in  the 
apex  of  the  left  lung  whose  tuberculous  or  syphilitic  nature  is 
left  in  doubt;  a  broad  milk-colored  patch  of  thickening  of 
the  capsule  of  the  liver.  It  is  to  be  noted  that  there  were  no 
gross  lesions  of  the  aorta. 


222  PUZZLES    AND    ERRORS 


On  the  classical  assumption  that  PARETIC  NEU- 
ROSYPHILIS  ("  general  paresis ")  is  a  fatal 
disease,  is  there  a  disease  PSEUD OPARETIC 
NEUROSYPHHIS  ("  pseudoparesis  ")  which  may 
recover  or  pursue  a  long  course  like  that  of  a  case 
of  diffuse  neurosyphilis  ("  cerebrospinal  syphilis  ")? 


Case  58.  Peter  Burkhardt  had  been  an  efficient  highway 
inspector,  but  in  his  forty-fifth  year  he  had  begun  to  be  unable 
to  do  his  work  satisfactorily.  His  wife  had  become  somewhat 
afraid  of  him.  He  had  had  somnolent  spells  in  his  chair  and 
had  squandered  money.  The  mental  symptoms  had  lasted 
for  some  six  months,  but  had  become  more  marked  during 
the  month  preceding  admission.  Burkhardt  would  at  times 
fail  to  recognize  his  friends. 

The  general  physical  condition  of  Burkhardt  was  very  good. 
The  pupils  were  irregular  and  reacted  sluggishly  both  to  light 
and  to  accommodation.  The  knee-jerks  and  ankle- jerks  were 
absent.  There  were  no  other  neurological  disorders  upon 
systematic  examination.  There  was  a  speech  defect.  Men- 
tally, little  could  be  determined  except  a  certain  sluggishness. 

History  and  physical  examination  at  once  suggested  gen- 
eral paresis.  The  serum  W.  R.  was  doubtful,  but  the  spinal 
fluid  reaction  was  positive,  as  was  the  gold  sol  reaction  (which 
was  "paretic");  the  globulin  and  albumin  were  greatly 
increased;  there  were  48  cells  per  cmm.  Antisyphilitic 
treatment,  consisting  of  salvarsan  twice  a  week  and  potassium 
iodid  by  mouth,  was  followed  by  a  rapid  mental  improve- 
ment. After  two  months,  Burkhardt  was  discharged  appar- 
ently normal,  with  all  the  blood  and  spinal  fluid  tests  neg- 
ative. He  has  been  taken  back  into  the  highway  service. 

I.  What  is  the  proper  definition  of  pseudoparesis?  Fournier 
termed  pseudoparesis  certain  cases  that  looked  like 
paresis  but  were  not  syphilitic  in  origin.  Of  these 
cases  the  most  characteristic  group  is  that  of  alcoholic 
pseudoparesis.  It  is  clear  that  there  will  be  no  diffi- 


PUZZLES   AND    ERRORS  223 

culty  in  the  definition  of  a  disease  pseudoparesis  whose 
entity  is  presented  in  the  adjective  that  precedes  the 
term  (e.g.,  alcoholic  pseudoparesis).  According  to  this 
usage,  a  case  of  pseudoparesis  would  be  one  in  which  the 
symptoms  and  possibly  some  of  the  signs  somewhat 
resemble  the  symptoms  of  paresis  itself  but  for  which 
another  etiology  could  be  fairly  established. 

2.  Are  there  any  cases  of  syphilitic  pseudoparesis?     We  are 

of  the  opinion  that  the  term  should  be  dropped.  It  is 
true  that  there  are  cases  which  clinically  look  like  gen- 
eral paresis  and  exhibit  the  appropriate  laboratory  signs 
of  general  paresis  but  seem  to  differ  from  paresis  in  their 
course  even  when  they  receive  no  treatment  whatever. 
In  the  present  phase  of  doubt  as  to  the  classification  of 
paretic  and  non-paretic  forms  of  neurosyphilis,  it  seems 
to  us  of  doubtful  utility  to  characterize  a  case  as  pseudo 
simply  because  it  differs  in  its  course,  particularly  as 
the  literature  has  always  duly  recognized  that  a  number 
of  cases  of  general  paresis  have  had  long  courses  and 
sometimes  very  long  remissions. 

There  is  also  another  group  of  cases  that  have  been 
termed  cases  of  pseudoparesis,  namely:  certain  cases  of 
neurosyphilis  which  clinically  look  like  general  paresis 
and  seem  to  be  following  its  classical  course  but  are 
interrupted  by  treatment.  Here  again  it  seems  to  us 
doubtful  whether  the  designation  pseudo  should  be 
attached  to  this  group  of  cases,  particularly  while  the 
whole  therapeutic  question  in  the  paretic  group  of  neuro- 
syphilis cases  remains  sub  judice.  Accordingly  we  are 
tempted  to  include  in  the  group  of  paretic  neurosyphilis 
cases  that  either  get  well  of  themselves,  or  get  well  under 
treatment,  or  pursue  a  very  long  course,  or  are  subject 
to  very  long  remissions.  But  we  make  this  decision  in 
terminology  without  prejudice  to  the  therapeutic  ques- 
tion and  it  is  open  to  any  critic  to  throw  these  cases  into 
an  atypical  non-paretic  group  of  neurosyphilis  cases. 

3.  How  shall  we  explain  the  absence  of  ataxia  of  case  Burk- 

hardt  when  knee-jerks  are  absent  and  when,  therefore, 
we  are  entitled  to  conclude  a  certain  degree  of  spinal 
disease?  As  stated  in  connection  with  case  Sullivan 
(16),  the  absence  of  knee-jerks  is  not  a  warrant  for 
terming  a  case  —  paresis  of  the  tabetic  form.  The  fact 
is  that  the  lesion  in  paresis  tends  to  be  intraspinal, 
just  as  the  higher  brain  lesions  tend  to  occur  within  the 
brain  substance.  The  meninges  are  relatively  spared 
both  within  the  cranium  and  within  the  spinal  canal. 


224  PUZZLES  AND   ERRORS 

The  characteristic  degeneration  of  posterior  nerve 
roots  which  we  find  in  tabes  dorsalis  is  not  necessarily 
found  in  general  paresis  even  when  there  are  somewhat 
extensive  spinal  lesions.  Accordingly  the  absence  of 
sensory  returns  by  way  of  the  posterior  nerve  roots 
which  characterizes  tabes  dorsalis  is  not  necessarily  a 
phenomenon  of  general  paresis.  The  mechanism  by 
which  the  knee-jerks  are  lost  depends  upon  histo- 
logical  detail.  They  may  be  lost  when  under  tabetic, 
conditions  the  posterior  roots  are  severely  diseased 
and  when  under  paretic  conditions  only  intraspinal 
collaterals  or  a  small  portion  of  fibres  are  affected. 
The  whole  question  hinges  upon  where  and  to  what 
degree  the  various  reflex  arcs  are  cut  in  the  disease. 
The  tabetic  phenomena  are,  as  so  commonly  stated, 
intradural ;  that  is,  the  sensory  or  gangliospinal  neurones 
at  certain  levels  are  affected  all  the  way  in  from  the 
points  at  which  they  pierce  the  dura  mater.  The  affec- 
tion of  these  and  other  neurones  in  general  paresis  is 
an  intraspinal  and  parenchymatous  affection. 


PUZZLES   AND   ERRORS  225 


Neurosyphilis ;  auditory  hallucinations;  ideas  of 
persecution ;  attacks  of  excitement.  SYPHILITIC 
PARANOIA  (Kraepelin)? 


Case  59.  Bridget  Curley  was  a  case  that  was  discharged 
from  the  Psychopathic  Hospital,  recovered,  after  26  days  in 
hospital.  The  symptoms  so  resembled  those  of  alcoholic 
hallucinosis  that  the  diagnosis  was  made  despite  the  fact  that 
the  patient  consistently  denied  the  use  of  intoxicants.  There 
was,  in  fact,  no  proof  that  she  drank  alcohol.  The  case  was, 
however,  not  clearly  one  of  alcoholic  hallucinosis  or  of  any 
other  well-defined  form  of  mental  disease.  A  provisional 
diagnosis  of  manic-depressive  psychosis,  manic  phase,  had,  in 
fact,  also  been  made. 

The  illness  had  begun  with  depression  and  inactivity, 
Bridget's  friends  accounted  for  these  conditions  on  the  ground 
that  a  lover  had  departed  for  Ireland.  A  few  days  after  the 
depression  began,  Bridget  became  dizzy  and  refused  to  give  a 
boarder  his  breakfast,  stating  that  she  had  lost  her  memory 
and  had  begun  to  hear  bells  ringing  and  people  talking.  She 
then  became  greatly  excited  and  was  brought  to  hospital, 
where  the  prolonged  baths  quieted  her. 

It  seems  that  Bridget  had  had  stomach  trouble  and  head- 
aches at  the  top  of  her  head  or  sometimes  in  her  temples. 
Physical  examination  showed  the  left  pupil  to  be  larger  than 
the  right,  a  slight  tremor  of  the  lips,  a  slight  systolic  murmur 
at  the  apex,  slightly  irregular  pulse,  and  moderate  edema  of 
ankles.  The  blood  serum  was  negative  to  the  W.  R.,  but 
lumbar  puncture  was  executed  and  the  fluid  showed  a  posi- 
tive W.  R. 

The  patient  was  tested  by  the  Binet  and  other  methods, 
and  although  35  years  of  age,  seemed  to  be  by  the  mental 
tests  hardly  over  II  years  old.  She  was  inclined  to  be  fe- 
verish, somewhat  restive,  and  pugnacious;  rather  slow  of 
speech,  sometimes  refusing  to  answer  and  grimacing.  Her 
pugnacity  was,  however,  easily  controllable,  and  the  excite- 


226  PUZZLES   AND    ERRORS 

ment  was  largely  at  night.     This  excitement  subsided  rapidly 
in  the  course  of  a  few  days. 

1.  What  is  the  diagnosis  in  this  case?     The  following  diag- 

noses and  suggestions  for  diagnosis  were  made  at  the 
staff  meetings: 

Unclassified  mania. 

Manic-depressive  psychosis,  manic  phase. 

Toxic  delirium. 

Dementia  praecox. 

Bacterial  infection  of  the  brain. 

Unclassified  delirium. 

Acute  delirium. 

Infectious  psychosis. 

Acute  confusional  psychosis. 

Psychopathic  personality  by  use  of  alcohol. 

Mental  deficiency  with  atypical  mental  state. 

Syphilitic  paranoia. 

2.  Is  this  a  case  of  syphilitic  paranoia?     The  so-called  syph- 

ilitic paranoia  of  Kraepelin  is  a  rare  and  uncertain  type 
of  syphilitic  mental  disease.  Delusions  and  hallu- 
cinations are  prominent.  As  a  rule,  the  onset  is  stated 
to  be  slow  and  insidious,  or  at  any  rate  there  are  a 
variety  of  indefinite  prodromata.  Jealousy  is  a  prom- 
inent feature,  sometimes  attended  with  marked  sexual 
excitement.  Auditory  hallucinations  and  ideas  of 
persecution  are  particularly  in  evidence.  The  most 
striking  feature  in  Kraepelin's  group  was  a  sudden  oc- 
currence and  equally  sudden  disappearance  of  violent 
excitement,  with  or  without  external  cause.  Thus, 
an  excitement  would  be  produced  by  a  few  words 
spoken,  and  immediately  after,  the  phase  of  excitement 
would  pass  and  the  patient  would  become  entirely 
friendly  and  accessible  once  more,  as  if  nothing  had 
happened.  About  half  of  Kraepelin's  cases  showed  a 
positive  serum  W.  R.  He  does  not  report  lumbar 
puncture  findings,  and  grounds  the  existence  of  disease 
upon  certain  autopsied  cases.  The  speech  and  writing 
disorder  of  paresis  as  well  as  the  characteristic  disori- 
entation  for  time  and  muscular  weakness  of  general 
paresis  were  absent  in  the  group.  It  appears  that 
most  cases  of  the  group  have  hitherto  been  placed  in 
dementia  praecox. 


PUZZLES   AND    ERRORS  227 


The  clinical  symptoms  of  CHRONIC  ALCOHOL- 
ISM are  sometimes  largely  identical  with  those  of 
PARETIC  NEUROSYPHILIS  ("general  paresis") : 
differentiation  by  means  of  the  laboratory  findings. 


To  demonstrate  this  proposition,  the  cases  of  Francis 
Murphy  (60)  and  David  Collins  (61)  are  in  point,  being 
sharp  foils  to  one  another. 

Case  60.  A  laboring  man  about  44  years  of  age  was  brought 
to  the  Psychopathic  Hospital  one  summer  day,  in  a  stupor. 
This  patient,  Francis  Murphy,  had  been  at  his  regular  work 
as  axeman  in  the  Park  Service,  when  he  suddenly  fell  in  a 
heavy  convulsion.  He  was  carried  to  a  general  hospital, 
still  in  convulsions,  and  ether  was  administered  to  quiet 
the  movements.  The  convulsions  shortly  ceased,  but  the 
patient's  consciousness  failed  to  clear;  hence  his  transfer  to 
the  Psychopathic  Hospital. 

Here  he  remained  much  disturbed  and  was  placed  in  a  room 
with  a  mattress  on  the  floor.  On  this  mattress  he  would 
crouch  on  all  fours  for  a  considerable  time,  looking  fixedly 
downward  as  if  at  an  object  on  the  floor,  unresponsive  to 
questions  but  compliant  with  efforts  to  place  him  on  his  back. 
He  gave  the  impression  of  daze  and  either  disorientation  or 
confusion. 

Within  twenty-four  hours  the  patient  became  more  tran- 
quil and  consciousness  became  clearer,  but  the  patient  was  at 
a  loss  to  bring  to  memory  either  recent  or  remote  events. 
However,  he  replied  to  questions,  giving  some  different  story 
each  time  he  was  approached.  Curiously  enough,  the  patient 
seemed  very  contented  and  good  natured  and  would  even 
laugh  foolishly  at  times,  saying  that  he  felt  fine  and  all 
ready  to  go  out  to  work. 

The  general  impression  conveyed  by  Francis  Murphy  at 
once  suggested  the  possibility  of  neurosyphilis.  Convul- 
sions, perhaps  initial  in  middle  age,  with  a  post-convulsive 


228  PUZZLES  AND   ERRORS 

stupor,  followed  by  a  partial  clearing  up,  with  persistent 
amnesia  and  a  suggestion  of  fabrications  with  euphoria,  bore 
out  the  suggestion. 

The  physical  examination  strengthened  the  impression 
of  neurosyphilis.  Well  developed  and  nourished,  florid,  with 
a  manual  tremor  and  sweating  of  the  palms,  the  patient 
was  in  general  without  physical  symptoms.  Neurologically, 
however,  whereas  the  left  pupil  was  larger  than  the  right  and 
reacted  properly  to  light,  the  right  pupil  was  a  bit  contracted, 
somewhat  irregular,  and  either  reacted  not  at  all  to  light  or 
very  slightly  so  (reacting  perfectly  to  accommodation).  The 
knee-jerks  could  be  obtained  only  with  reinforcement,  and 
several  other  reflexes  could  not  be  elicited  (triceps,  radial, 
ulnar,  periosteal,  Achilles,  umbilical).  Moreover,  the  heel-to- 
knee  test  was  poorly  performed;  some  of  the  common  tests 
phrases  were  very  poorly  repeated ;  there  was  marked  tremor 
in  writing;  and  the  paragraphia  seemed  to  be  not  merely 
peripheral,  for  syllables  were  left  out  in  words  and  ordinary 
words  spelled  incorrectly  (psychographic  disturbance). 

We  do  not  care  here  to  insist  that  the  right  pupil  was  really 
an  example  of  the  Argyll-Robertson  phenomenon  since  the 
slightest  tinge  of  doubt  is  important  if  a  positive  diagnosis 
is  practically  equivalent  to  asserting  syphilis.  Practically, 
however,  the  right  pupil  was  regarded  as  an  Argyll- Robertson 
pupil  under  hospital  conditions  (flash-light  reaction).  Argyll- 
Robertson  pupil,  areflexia,  speech  disorder,  writing  disorder, 
memory  disorder,  conduct  disorder,  and  euphoria,  all  with  a 
history  of  convulsions,  certainly  warranted  the  tentative  diag- 
nosis of  neurosyphilis. 

As  usual,  resort  was  made  to  the  W.  R.  in  the  serum  and  in 
the  spinal  fluid.  One  of  the  first  results  to  come  through 
from  the  laboratory  was  the  absence  of  globulin,  normal 
albumin,  negative  gold  sol  reaction,  and  a  cell  count  of  two 
cells  per  cmm.  in  the  spinal  fluid.  Later  the  W.  R.'s  were 
returned  negative  for  blood  and  spinal  fluid. 

In  the  meantime,  an  illuminating  change  had  occurred  in 
the  patient,  for  two  days  later,  —  three  days  after  the  first 
convulsion  in  the  park,  —  the  patient  had  apparently  quite 
recovered;  his  consciousness  became  nearly  clear;  he  could 


PUZZLES  AND   ERRORS  229 

remember  every  event  up  to  the  time  of  the  convulsion,  and 
his  memory  came  back  in  appropriate  degree  for  both  remote 
and  recent  events. 

The  patient,  it  appeared,  had  for  some  time  been  drinking 
more  and  more  heavily.  In  recent  days,  he  had  been  taking 
five  or  six  whiskeys  and  a  half  dozen  beers  daily  on  the 
average,  and  often  much  more.  About  ten  years  before,  the 
patient  narrated,  there  had  been  a  convulsion  at  a  ballgame, 
and  this  convulsion  the  patient  himself  called  a  "  rum  fit." 

Here,  then,  is  a  case  of  ALCOHOLIC  PSEUDOPARESIS.  With- 
out the  W.  serum  test  and  without  the  spinal  fluid  examina- 
tion, it  is  probable  that  the  diagnosis  of  general  paresis  might 
have  clung  to  the  patient  for  some  time  on  account  of  the 
apparent  Argyll- Robertson  pupil,  which  had  to  be  accepted 
as  such  on  the  flash-light  data.  In  point  of  fact,  in  this  case 
the  pupil  later  reacted  more  normally  to  light,  and  the  speech 
and  writing  disorders  measurably  cleared  up. 

1.  Can  alcohol  produce  the  Argyll- Robertson  pupil?    The 

majority  of  neurologists  would  today  answer,  Yes. 

2.  If  in  the  case  of  Francis  Murphy,  the  W.  R.  in  the  blood 

had  happened  to  be  positive  on  account  of  a  non-neural 
syphilitic  infection  (spinal  fluid  negative),  would  the 
diagnosis  general  paresis  be  warranted?  Probably  the 
diagnosis  general  paresis  would  have  been  made.  If 
the  patient  had  been  lost  to  observation,  he  might 
well  have  been  regarded  as  an  atypical  paretic  with 
prodromal  convulsions. 

3.  Would  positive  globulin  and  excess  albumin  in  the  spinal 

fluid  alone  or  in  association  with  a  positive  serum  W.  R. 
warrant  the  diagnosis  general  paresis  or  neurosyphilis? 
The  chances  are  that  most  neurologists  would  advocate 
proceeding  to  treatment  in  any  case  of  positive  serum 
reaction,  whether  or  not  there  was  globulin  or  excess 
albumin;  but  the  positive  globulin  and  excess  albumin 
would  probably  not  warrant  the  diagnosis  general 
paresis  or  neurosyphilis  in  the  absence  of  excess  cells 
and  the  characteristic  gold  sol  reaction  and  W.  R.  in 
the  fluid. 

4.  Is  the  case  of  Francis  Murphy  one  of  alcoholic  epilepsy 

(as  suggested  by  Murphy's  own  phrase,  "  rum  fits  ")? 
It  must  be  remembered  that  epileptics  become  alco- 
holic and  that  epileptic  convulsions  increase  or  become 


230  PUZZLES   AND   ERRORS 

more  severe  with  alcoholism.  On  the  other  hand,  the 
literature  indicates  that  alcoholism  can  produce  con- 
vulsions, as  can  many  other  factors.  The  literature 
also  indicates  that  there  is  a  condition  of  epilepsy  in 
which  the  convulsive  tendency  sets  in  as  a  result  of 
alcoholism  in  a  patient  not  previously  disposed  to  epi- 
lepsy; it  appears  also  that  sometimes,  though  very 
rarely,  the  epilepsy  continues  after  withdrawal  of  alcohol, 
and  even  after  giving  up  the  habit.  Francis  Murphy 
appears  to  have  had  but  two  spells  of  convulsions,  both 
of  them  following  heavy  bouts  with  alcohol.  There  is 
so  far,  then,  no  warrant  for  calling  Francis  Murphy's 
case  one  of  alcoholic  epilepsy. 

5.  Does  the  use  of  alcohol  by  a  subject  destroy  the  value 
of  the  W.R.?  It  has  been  held  by  some  that  alcohol- 
ism interferes  with  the  accuracy  of  the  W.  R.  This 
has  not  been  our  experience  and  for  the  present  we 
are  of  opinion  that  the  results  have  the  same  value 
in  alcoholics  as  in  non-alcoholics.  The  next  case 
(Collins,  61)  is  one  in  which  a  positive  W.  R.  occurred 
in  an  alcoholic.  When  dealing  with  paretic  neuro- 
syphilis  it  is  especially  true  that  the  W.  R.  is  disturbed 
very  rarely,  if  at  all,  by  toxins  or  drugs,  except  anti- 
syphilitic  drugs. 


PUZZLES   AND   ERRORS 


231 


ALCOHOLISM  may  cloud  the  diagnosis  of  NEURO- 
SYPHILIS.     Differentiation  by  laboratory  tests. 


Case  61.  David  Collins  was  a  steamfitter  of  about  43 
years  of  age,  picked  up  at  6.45  a.m.  in  the  midst  of  convulsions 
and  talking  incoherently,  in  a  state  apparently  of  fairly  clear 
consciousness.  On  arrival  at  the  hospital,  the  patient  was 
able  to  tell  how  he  had  always  been  a  hard  drinker,  and  how 
during  the  past  week  of  unemployment  he  had  taken  large 
quantities  of  poor  whiskey,  —  perhaps  an  average  of  a  pint  a 
day.  Collins  also  told  how  he  had  had  delirium  tremens  sev- 
eral times,  but  he  said  the  present  spell  was  quite  unlike  de- 
lirium tremens.  There  was  no  disorientation  or  impairment  of 
memory,  and  the  patient  did  not  in  any  wise  suggest  a  mental 
case  a  few  hours  after  admission. 

It  appears,  according  to  Collins,  that  he  had  obtained  some 
work  the  night  before,  and  had  quit  work  about  6.30,  where- 
upon he  stepped  into  a  barroom,  took  one  drink  of  whiskey, 
left  the  barroom,  walked  down  the  street,  and  suddenly  lost 
track  of  the  world,  coming  to  consciousness  in  a  carriage  with 
two  policemen,  but  remaining,  as  he  said,  "dopy,"  inatten- 
tive, and  confused.  After  a  meal,  however,  the  patient  began 
to  feel  better  and  soon  felt  quite  all  right. 

The  physical  examination  was  quite  negative  except  that 
neurologically  there  was  lingual  and  manual  tremor,  a  speech 
defect,  apparent  only  with  test  phrases,  unsteadiness  of  hand- 
writing, left  knee-jerk  greater  than  right,  a  left-sided  Babinski 
reflex,  and  a  difficulty  in  executing  rapid  successive  move- 
ments (dysdiadochokinesis) .  This  degree  of  neurological  dis- 
order in  our  experience  warrants  lumbar  puncture  as  well  as  a 
serum  test.  The  lumbar  puncture  shortly  disclosed  a  positive 
globulin  and  excess  albumin,  and  the  returns  from  the  W.  R.'s 
were  positive  for  both  spinal  fluid  and  blood  serum.  The 
data  of  the  gold  sol  reaction  were  not  available  on  account  of 
technical  difficulties.  However,  it  appears  that  the  diagnosis 
of  neurosyphilis  could  hardly  be  avoided  in  this  case. 


232  PUZZLES  AND   ERRORS 

David  Collins  differs  from  Francis  Murphy,  then,  in  show- 
ing a  positive  blood  and  spinal  fluid  reaction  for  syphilis  as 
well  as  a  positive  globulin  and  excess  albumin.  As  above 
remarked,  it  is  probable  that  the  positive  globulin  and  excess 
albumin  would  not  warrant  more  than  a  suspicion  of  neuro- 
syphilis  taken  by  themselves. 

Unfortunately,  we  were  unable  to  persuade  the  patient  to 
submit  to  treatment,  and  from  the  patient's  point  of  view 
possibly  his  decision,  not  to  submit  to  treatment,  was  a  good 
one  since  he  has  had  no  symptoms  of  any  sort  for  a  period  of  18 
months  since  his  episode.  However,  as  abundantly  else- 
where demonstrated,  we  feel  that  the  patient  is  wrong,  and 
that  the  physicians  are  right  in  urging  treatment. 

1.  Is  not  the  convulsive  episode  an  alcoholic  phenomenon 

in  David  Collins  entirely  separate  from  the  patient's 
general  and  neurosyphilis?  Possibly;  however,  an 
outbreak  of  neurological  symptoms  with  spontaneous 
recovery  is  not  only  consistent  with  the  diagnosis  of 
syphilis,  but  somewhat  characteristic  of  neurosyphilis. 
We  suspect  that  another  attack  will  occur  in  David 
Collins.*  We  shall  from  time  to  time  make  use  of  the 
social  service  to  suggest  his  going  under  treatment,  and 
shall  employ  his  record  of  contact  with  a  public  institu- 
tion to  drive  in  our  suggestion.  Still  it  is  clear  that 
there  are  numerous  cases  in  the  community  that  are 
not  accessible  to  social  service  initiated  from  a  public 
institution.  Accordingly,  educational  propaganda  is 
necessary  for  salvage  of  the  middle-  and  upper-class 
victims  of  syphilis.  It  is  a  little  unfortunate  that  the 
ethics  of  the  private  practitioner  make  such  salvage  of 
middle-  and  upper-class  persons  not  very  likely.  Might 
it  not  be  that  an  extension  of  state  medicine  to  this 
field  would  incidentally  increase  the  amount  of  success- 
ful private  practice? 

2.  What  may  be  the  cause  of  such  a  convulsive  episode  as 

that  of  David  Collins?  It  would  appear  that  the  con- 
vulsions of  general  paresis  and  of  neurosyphilis  in  gen- 
eral often  occur  without  gross  structural  lesions  of  the 
brain.  It  may  be  suggested  that  vascular  irritation  or 

*  Since  this  was  written  Collins  has  had  further  difficulties 
related  to  his  neurosyphilis,  improving  under  treatment. 


PUZZLES  AND   ERRORS  233 

parenchymal  irritation  by  spirochetes,  acting  in  appro- 
priate parts  of  the  central  nervous  system,  can  produce 
such  convulsions. 

3.  What  is  the  significance  of  the  unilateral  phenomenon  in 

David  Collins  (left  knee-jerk  greater  than  right;  left- 
sided  Babinski)?  The  current  explanation  of  hyper- 
reflexia  is  that  somehow  inhibitory  impulses  from  upper 
portions  of  the  nervous  system  have  ceased  to  influence 
the  local  arcs  that  mechanize  reactions  like  the  knee-jerk 
and  the  normal  plantar  reflex.  The  phenomena  are 
commonly  found  in  cases  with  pyramidal  tract  disorder, 
and  in  the  case  of  David  Collins  one  may  suspect,  there- 
fore, that  there  was  a  central  disorder  affecting  the  right 
pyramidal  tract  above  its  decussation.  One  might 
suspect  that  the  convulsions  were  initiated  by  a  lesion 
(whether  gross  or  microscopic  in  range)  in  the  right 
side  of  the  cerebrum ;  but  whether  in  the  white  matter 
or  in  the  gray  matter  must  be  left  doubtful.  The 
clearing  up  of  all  symptoms  suggests  either  that  the 
lesion  was  microscopic  in  range  or  that  the  phenomena 
were  transient  and  functional. 

4.  Can  the  dysdiadochokinesis  be  used  to  indicate  cerebellar 

lesion  in  David  Collins?  Possibly;  but  it  does  not 
appear  that  the  difficulty  in  executing  successive  move- 
ments was  unilateral.  It  seems  impossible  to  bring 
into  close  topographical  relation  the  basis  for  the 
Babinski  and  left-sided  hyperreflexia,  and  the  basis  for 
the  dysdiadochokinesis.  Alcohol  is  sometimes  asserted 
to  exert  an  especial  effect  upon  the  cerebellum. 

5.  Must  we  suppose  structural  lesions,  either  (a)   of  the 

nature  of  cell  losses  demonstrable  microscopically,  or 
(b)  of  the  nature  of  secondary  degenerations  demon- 
strable by  Weigert  myelin  sheath  methods,  in  the  case 
of  David  Collins?  It  appears  that  we  do  not  need  to 
assert  the  existence  of  such  lesions. 

6.  Could  the  hyperreflexia  and  the  Babinski  reaction  be  due 

to  local  spinal  cord  disease  ?  Possibly ;  but  the  existence 
of  other  neurological  symptoms  (lingual  and  manual 
tremor,  speech  defect  to  test  phrases,  ataxic  hand- 
writing, and  dysdiadochokinesis)  makes  it  probable 
that  there  were  lesions,  or  at  any  rate  disordered  func- 
tions, within  the  cranium;  and  there  appears  to  be  no 
basis  for  asserting  local  spinal  cord  disease. 


234  PUZZLES  AND   ERRORS 


Differential  diagnosis  between  NEURO SYPHILIS 
and  ACUTE  ALCOHOLIC  PSYCHOSIS. 


Case  62.  Joseph  Buck  was  a  chef  of  60  years  who  came  in, 
seeking  advice  because  his  memory  was  getting  poor;  he 
was  unable  to  remember  names  and  what  he  was  about  to  do. 
He  was  tremulous  and  had  much  pain  in  his  limbs.  He  had 
been  drinking  heavily  for  weeks,  —  probably  ten  weeks; 
in  fact,  he  described  himself  as  having  had  "  the  shakes  " 
and  as  having  lately  seen  animals  and  people  that  were  unreal. 
He  had  had  the  shakes  before  and  the  condition  had  lasted 
for  two  to  three  days  after  alcohol  was  discontinued. 

Physically,  Buck  was  tall,  well  developed,  although  poorly 
nourished,  with  a  skin  suggesting  alcoholism.  There  was 
a  slight  acne  over  the  back  and  chest;  there  was  a  slight 
enlargement  of  the  heart,  with  blood  pressure,  systolic,  180, 
diastolic,  120.  There  was  a  corneal  opacity  of  the  left  eye, 
which  the  patient  said  was  the  result  of  syphilis  following  a 
chancre,  which  he  had  acquired  at  the  age  of  27.  There 
was  also  a  ptosis  of  the  upper  lid  of  the  left  eye.  The  right 
pupil  was  irregular  and  reacted  to  light  sluggishly,  and  with 
a  very  small  excursion.  The  patient  was  slightly  deaf  in 
both  ears.  The  deep  reflexes  were  all  lively  and  equal.  The 
tremor  was  most  marked  in  finely  coordinated  movements. 
There  was  a.  slight  swaying  in  the  Romberg  position  but  the 
sign  could  not  be  said  to  be  present.  The  gait  was  unsteady. 
There  was  a  marked  tenderness  over  the  nerve  trunks. 

So  far  as  mental  examination  went,  it  seemed  that  the 
patient's  claim  of  amnesia  was  subjective.  There  was  cer- 
tainly no  more  amnesia  than  a  slight  difficulty  in  recalling 
details.  The  diagnosis  of  alcoholism  with  convalescence 
from  delirium  tremens  would  certainly  seem  to  have  been 
sufficient  for  the  phenomena,  and  the  suggestion  of  alcoholic 
neuritis  only  confirmed  the  picture.  To  be  sure,  one  might 
expect  a  diminution  or  absence  of  deep  reflexes;  still,  these  re- 
flexes may  be  over-active  in  an  irritative  stage  of  the  disease. 


PUZZLES   AND   ERRORS  235 

Naturally,  however,  the  history  of  syphilis  and  the  pupil- 
lary phenomena  and  ptosis,  made  the  consideration  of  neuro- 
syphilis  necessary.  Both  serum  and  fluid  W.  R.'s  proved 
positive;  there  was  an  excessive  amount  of  albumin  and 
globulin,  the  gold  sol  reaction  was  typically  "  paretic,"  and 
there  were  377  cells  per  cmm. 

The  patient  improved  upon  a  rest  treatment  and  was 
given  injections  of  mercury  for  his  syphilis.  After  a  few 
months  he  felt  well  enough  to  return  to  work,  and  continued 
at  work  throughout  a  season,  receiving  mercurial  treatment 
throughout  this  time.  A  spinal  fluid  examination  fifteen 
months  later  showed  a  weaker  gold  sol  reaction,  reduction 
in  the  amount  of  globulin  and  albumin,  and  but  26  cells  to 
the  cmm.  The  W.  R.'s  had  remained  positive. 

1.  What  are  the  forms  of  syphilitic  neuritis?     According  to 

Nonne,  syphilitic  neuritis  and  polyneuritis  have  at 
last  acquired  standing  in  neuropathology.  The  older 
claims  depended  upon  findings  on  palpation  and  re- 
covery after  antisyphilitic  treatment.  Since  the  in- 
troduction of  salvarsan,  cases  of  ophthalmoplegia, 
facial,  acoustic,  and  optic  nerve  disease,  as  well  as 
neuritis  of  the  extremities,  have  been  reported  in  large 
numbers.  These  phenomena  are  to  be  regarded  as 
neurorecidives  in  the  modern  sense  of  that  term.  The 
neurorecidive  is  not  a  salvarsan  effect,  but  is  an  effect 
of  the  syphilitic  process  itself,  settling  in  the  peripheral 
nerves.  Paresthesias  are  especially  prominent  in  periph- 
eral mono-  or  polyneuritis,  and  this  point  is  of  some 
value  in  differentiating  the  syphilitic  peripheral  neuritis 
from  root  neuritis.  Root  neuritis  is  more  often  charac- 
terized by  neuralgic  attacks.  Objective  hyperesthesia 
of  neuromuscular  origin  is  also  found  in  these  cases, 
demonstrated  by  pressure  on  the  nerves.  The  motor 
phenomena  consist  in  a  flaccid  paresis  or  paralysis, 
especially  affecting  the  radial,  ulnar,  and  peroneal 
nerves.  Nonne  states  that  it  is  rare  for  syphilis  to 
affect  a  single  nerve  region,  and  he  regards  cases  in 
which  a  single  region  alone  is  affected  as  usually  due  to 
a  local  gummatous  process. 

2.  What  is  the  significance  of  377  cells  per  cmm.?     See 

discussion  of  Washington  (Case  66). 


236  PUZZLES   AND   ERRORS 


Differential  diagnosis  between  NEUROSYPHILIS 
and  CHRONIC  ALCOHOLISM. 


Case  63.  Albert  Fielding,  46,  was  an  insurance  broker, 
who  was  brought  to  the  hospital  for  excessive  alcoholism. 
Indeed,  he  showed  all  the  signs,  both  of  chronic  and  acute 
intoxication,  except  that  there  was  no  nerve  trunk  tenderness. 
Fielding  was  very  loquacious  though  his  speech  was  rather 
thick.  He  showed  tremor  of  hands  and  an  alcoholic  skin. 
Physical  and  neurological  examination  proved  entirely  neg- 
ative. 

Fielding  claimed  that  he  had  had  a  nervous  breakdown  at 
about  36  years  of  age,  after  disappointment  in  love.  He  had 
the  drinking  habit  and  began  to  drink  more  and  more.  He 
had  now  become  nervous  and  tremulous  and  had  to  drink  in 
order  to  brace  himself.  After  a  few  days,  the  patient  began 
to  be  much  better,  having  recovered  from  acute  alcoholism. 
Mental  examination  now  showed  good  memory  with  orienta- 
tion intact.  There  was  a  certain  tendency  to  reminiscence 
and  to  somewhat  childish  actions.  He  had  attempted  to  stop 
drinking  but  had  been  unable  to  quit.  As  a  matter  of  fact, 
his  mother  and  father  had  been  excessive  drinkers  and  he  had 
inherited  the  tendency,  etc. 

The  diagnosis  seemed  to  be  plain.  The  routine  W.  R. 
upon  the  blood  serum  was  negative.  However,  the  patient 
had  remarked  during  the  history  taking,  that  he  had  had  a 
chancre  and  secondary  symptoms  of  syphilis.  Accordingly, 
lumbar  puncture  was  resorted  to.  The  fluid  showed  a  slightly 
positive  W.  R.;  the  gold  sol  reaction  was  of  the  syphilitic 
type;  there  was  a  considerable  increase  in  albumin  and 
globulin,  and  there  were  20  cells  per  cmm.  The  diagnosis 
of  neurosyphilis  seemed  clear. 

Course :  The  patient  received  six  months'  treatment  in  a 
sanatorium  but  the  symptoms  remained  almost  as  before, 
and  the  patient  showed  the  same  childishness  and  inability 
to  take  care  of  himself.  Since  the  symptoms  continued  six 


PUZZLES   AND   ERRORS  237 

months  after  the  withdrawal  of  alcohol,  it  might  well  be 
suspected  that  the  condition  was  more  than  a  merely  alco- 
holic one.  However,  in  a  number  of  purely  alcoholic  cases, 
such  long-standing  effects  are  found:  even  as  long  as  six 
months  or  longer  after  the  withdrawal  of  the  alcohol,  and  one 
might  conclude  therefore  that  Fielding  was  actually  a  victim 
of  alcoholic  dementia.  The  spinal  fluid  after  these  six  months 
(during  which  period  antisyphilitic  treatment  was  given) 
showed  no  change,  and  the  prognosis  was  offered  that  the 
case  would  probably  develop  into  one  of  paresis. 

A  year  later,  after  six  months  sanatorial  care  and  six 
months  life  in  the  community,  the  patient  returned  to  the 
Psychopathic  Hospital  in  an  alcoholic  condition.  The  lumbar 
puncture  showed  all  signs  negative  except  the  W.  R.  which 
was  slightly  positive.  The  W.  R.  of  the  blood  was  negative. 

In  connection  with  this  case,  see  the  case  of  paresis  sine 


What  is  the  relation  of  the  syphilitic  and  alcoholic  process 
in  Robert  Fielding?  One  does  not  like  to  break  the 
so-called  rule  of  parsimony  in  diagnosis,  but  it  would 
seem  that  the  effects  in  Fielding  are  the  combined 
effects  of  syphilis  and  alcoholism. 


238  PUZZLES   AND   ERRORS 


Differential  diagnosis  between  NEURO SYPHILIS, 
DIABETIC  PSEUDOPARESIS  and  BRAIN 
TUMOR. 


Case  64.  A  large  and  imposing  person,  Calvin  Hall,  55, 
had  been  employed  as  a  doorkeeper  and  guard,  in  which 
position  he  was  on  duty  for  12  to  14  hours  daily.  Eventually, 
however,  he  had  begun  to  have  a  good  deal  of  pain  in  the  legs 
and  a  few  months  before  observation,  one  day,  his  legs  gave 
way  and  he  fell  to  the  floor.  There  was,  however,  no  loss  of 
consciousness,  and  he  was  carried  to  a  general  hospital.  The 
result  of  an  examination  there  was  that  his  family  was  in- 
formed that  he  had  some  nervous  trouble. 

Hall  now  began  to  be  melancholy  and  wept  a  good  deal. 
His  appetite  and  sleep  remained  intact.  He  felt  too  weak  to 
walk.  At  the  end  of  about  a  year,  he  began  to  improve  and 
again  became  able  to  do  a  little  light  work.  About  a  month 
before  coming  to  the  Psychopathic  Hospital,  about  two  years 
after  the  onset  of  symptoms,  Hall  suddenly  began  to  talk 
excessively,  in  a  rambling  and  rather  senseless  way.  A  fort- 
night later,  he  began  to  suffer  from  insomnia  and  restlessness. 

Some  medical  facts  were  available:  It  seems  that  at  25 
years  this  patient  had  become  infected  with  syphilis  though 
there  had  never  been  any  secondary  signs.  He  was  married 
four  years  later  but  there  had  not  been  any  children.  More- 
over, for  four  years  past,  the  patient  had  been  treated  for 
glycosuria. 

Upon  admission,  the  patient's  sensorium  was  clear,  but 
his  orientation  was  only  partial.  He  could  give  a  fair  account 
of  his  life,  but  it  appeared  that  his  memory  was  somewhat 
impaired.  There  were  auditory  hallucinations  (voices  of 
relatives).  He  often  mistook  the  identity  of  persons  about 
him.  He  talked  in  a  grandiose  fashion  of  his  great  strength 
and  especially  of  a  God-given  power  to  read  minds.  His 
flow  of  thought  was  rapid,  rambling,  circumstantial,  and  with 
traces  of  irrelevance.  He  was  rather  continuously  busy  and 


PUZZLES   AND   ERRORS  239 

at  times  restive.  There  was  a  good  deal  of  emotional  agita- 
tion and  apprehensiveness,  and  again  the  patient  would 
become  suspicious  and  tearful. 

Physically,  there  was  a  discharging  sinus  connected  with 
the  right  humerus,  close  to  the  elbow.  The  pupils,  though 
equal  and  regular,  were  sluggish  in  reaction  to  light.  The 
knee-jerks  and  ankle- jerks  were  absent.  There  was  no  Rom- 
berg  sign  but  there  was  some  swaying  in  the  Romberg  position. 
There  was  a  moderate  ataxia  in  walking.  Glycosuria  to  a 
moderate  degree  was  determined.  There  were  no  casts  or 
albumin  in  the  urine.  The  W.  R.  of  the  blood  and  of  the 
spinal  fluid  was  negative.  The  albumin  of  the  fluid,  however, 
was  considerably  increased.  X-ray  examination  of  the  skull 
yielded  a  suggestion  of  absorption  of  the  posterior  clinoid 
processes  of  the  sella  turcica.  The  X-ray  examination  of 
the  arm  in  the  region  of  the  sinus  showed  a  chronic  os- 
teomyelitis, possibly  syphilitic  (or  diabetic?). 

The  diagnostic  problems  in  the  case  of  Calvin  Hall  are 
extremely  intricate.  There  are  clinical  suggestions  of  general 
paresis,  not  confirmed  by  the  laboratory  findings. 

1.  Are  we  dealing  with  a  case  of  diabetic  pseudoparesis? 

Is  the  pain  in  the  legs  of  like  origin,  and  has  a  neuritic 
process  led  to  the  absence  of  the  knee-jerks?  The 
Allen  treatment  appears  to  have  had  no  beneficial 
result  in  this  case. 

2.  Is  there  a  tumor  of  the  sella  region,  which  could  account 

for  the  mental  symptoms  and  the  glycosuria?  The 
spinal  fluid  albumin  might  be  regarded  as  consistent 
with  a  variety  of  psychoses,  including  that  of  brain 
tumor.  We  have  to  remember  the  definite  history  of 
infection,  the  sterile  marriage,  and ^the.  possibly  syphi- 
litic osteomyelitis. 


240  PUZZLES   AND   ERRORS 


DIABETES  AND  NEURO  SYPHILIS,  relations? 


Case  65.  Donald  Barrie,  a  man  of  61,  diabetic  for  several 
years,  had  begun  to  worry  about  the  diabetes,  feeling  that 
he  was  about  to  die,  and  had  gone  so  far  as  to  make  several 
threats  of  suicide.  Hence  he  was  brought  to  the  Psycho- 
pathic Hospital  for  observation. 

Barrie  was  rather  well  developed  and  nourished,  although 
he  looked  far  older  than  he  was.  There  was  a  marked  arcus 
senilis;  the  skin  was  dry  and  rough;  the  radial  and  other 
accessible  vessels  were  markedly  sclerosed;  abdomen  obese; 
right  testicle  very  low  with  thickened  and  hard  epididymis. 

Neurologically  there  was  little  abnormal  to  discover.  The 
pupils  were  irregular;  both  reacted  fairly  well  to  light. 
There  was  a  slight  tremor  of  the  extended  hands,  and  still 
less  of  the  tongue.  The  voice  was  slightly  thick  and  the 
patient  stumbled  somewhat  on  test  phrases.  Urine:  specific 
gravity,  1029;  sugar;  no  acetone;  no  diacetic  acid.  Sugar  2 
to  II  grams  for  24  hours  on  ordinary  diet.  It  proved  im- 
possible to  get  the  patient  sugar-free,  either  by  cutting  down 
the  carbohydrates  or  by  using  the  Allen  method. 

Mentally,  the  depression  with  reiteration  of  wrong-doing 
and  self-accusation  because  of  the  contraction  of  syphilis, 
were  the  striking  features.  There  was,  to  be  sure,  a  slight 
imperfection  of  memory  for  remote  events ;  memory  for  recent 
events  and  knowledge  of  current  events  was  very  poor. 
Barrie  claimed  that  his  condition  was  entirely  hopeless,  that 
his  memory  was  exceedingly  bad,  and  that  he  was  no  longer 
capable  of  supporting  his  family. 

I.  What  shall  be  said  as  to  diagnosis  in  a  man  of  61  with 
glycosuria,  depression,  amnesia,  sluggish  pupil,  slight 
tremor,  slight  speech  defect,  and  a  history  of  syphilis? 
The  W.  R.  of  the  serum  proved  positive,  and  also  the 
W.  R.  of  the  spinal  fluid.  The  gold  sol  reaction  of  the 
fluid  was  of  the  syphilitic  type.  There  were  112  cells 


PUZZLES  AND   ERRORS  24! 

per  cmm.,  there  was  an  excess  of  albumin,  and  a  large 
amount  of  globulin.  Accordingly,  the  diagnosis  of 
PARETIC  NEUROSYPHILIS  ("  general  paresis "),  espe- 
cially^in  view  of  the  laboratory  findings,  seems  necessary. 

2.  What  is  the  cause  of  the  glycosuria?     First:   possibly 

it  has  no  relation  with  the  syphilis;  secondly:  it  may 
possibly  be  due  to  a  syphilitic  involvement  of  the  pan- 
creas; thirdly:  it  is  barely  possible  that  it  is  due  to 
syphilitic  disease  of  the  fourth  ventricle  or  of  the  base 
of  the  brain,  involving  the  pituitary  region.  Perhaps 
our  case  is  too  complex  for  analysis.  At  all  events,  the 
case  brings  up  the  possibility  of  a  syphilitic  glycosuria. 

3.  Can  the  diabetes  in  the  case  of  Barrie  be  explained  as 

syphilitic?  Warthin  of  Ann  Arbor  has  recently  de- 
scribed somewhat  remarkable  spirochete  findings  in 
his  autopsy  material.  The  order  of  organic  infection 
according  to  frequency  is:  aorta,  heart,  testis,  adrenal, 
pancreas,  nervous  system,  liver,  and  spleen.  Warthin 
has  called  attention  to  the  relation  of  pancreatitis  and 
spirochetosis  to  diabetes  in  a  recent  review  *  of  findings 
in  41  autopsied  cases  from  the  University  Hospital  in 
Michigan.  Warthin  found  active  luetic  lesions  in  the 
pancreas  in  6  cases. 

*  Warthin:  "  Persistence  of  active  lesions  and  spirpchetes 
in  the  tissues  of  clinically  inactive  or  '  cured '  syphilitics," 
American  Journal  of  Medical  Sciences,  CLII,  1916. 


242  PUZZLES   AND   ERRORS 


Hemianopsia  in  a  case  of  neurosyphilis. 


Case  66.  Lawrence  Washington,  a  colored  cabman,  58 
years  of  age,  began  to  forget  addresses  given  him  by  his  fares. 
Moreover,  he  could  no  longer  see  as  well  as  before,  especially 
on  looking  toward  the  right  side.  He  himself  states  that  the 
visual  trouble  dated  back  as  long  ago  as  his  39th  year,  at 
which  time  he  had  a  terrific  pain  in  both  temples,  leading 
back  from  the  eyes.  Washington  thought  that  his  vision 
had  been  getting  slowly  but  steadily  worse  ever  since. 

We  got  the  impression  that  the  amnesia  claimed  by  Wash- 
ington was  more  or  less  subjective  and  he  was  found  to  be 
well  informed.  This  association  of  amnesia  and  impairment 
of  vision  naturally  suggests  syphilis.  The  patient  himself 
stated  that  he  had  had  a  chancre  at  the  age  of  18. 

We  found  the  W.  R.  of  the  serum  to  be  appropriately  pos- 
itive. The  W.  R.  of  the  spinal  fluid  was  also  positive  though 
weakly  so.  There  was  an  excess  of  albumin;  globulin  ap- 
peared in  large  amount ;  the  gold  sol  reaction  was  of  the  syph- 
ilitic type;  there  were  186  cells  in  the  spinal  fluid. 

Is  this  case  one  of  paresis  or  of  some  other  form  of  cerebro- 
spinal  syphilis?  Let  us  consider  the  data  of  the  physical 
examination.  On  the  whole,  the  patient  was  well  preserved. 
There  was  a  slight  radial  arteriosclerosis,  but  on  the  whole 
the  cardiovascular  system  was  almost  negative.  The  blood 
pressure  was  100  systolic,  65  diastolic.  Neurologically  the 
visual  field  of  the  left  eye  was  somewhat  limited,  and  there 
was  a  temporal  hemianopsia  of  the  right  eye.  The  ophthal- 
moscopic  examination  showed  a  disseminated  choroiditis  on 
both  sides.  The  right  pupil  failed  to  react  to  light.  The  left 
pupil  reacted  slowly.  Both  pupils  reacted  properly  to  accom- 
modation. 

The  knee-jerks  could  be  obtained  only  on  reinforcement, 
and  when  obtained,  the  right  was  apparently  more  active 
than  the  left.  The  left  Achilles  was  absent ;  the  right  present. 
There  were  no  other  abnormal  reflexes. 


PUZZLES   AND   ERRORS  243 

The  motility  of  the  facial  muscles  was  somewhat  impaired. 
Finger-to-finger  and  finger-to-nose  tests  were  rather  poorly 
done.  The  muscle  sense  was  good;  there  was  no  swaying 
in  Romberg  position ;  and  there  was  no  speech  defect. 

We  are  unable  to  decide  whether  the  case  is  one  of  the 
parenchymatous  type  (paretic)  or  of  the  meningovascular 
type  of  neurosyphilis.  It  is  certainly  rather  unusual  to  find 
hemianopsia  in  a  paretic. 

We  have  been  unable  to  get  definite  results  from  the  treat- 
ment of  this  case,  since  the  patient  would  not  return  for 
months  after  getting  an  injection  or  two  of  salvarsan,  on  the 
ground  that  he  was  improved  enough  and  did  not  require 
further  treatment. 

I.  What  conclusion  can  be  drawn  from  the  186  cells  per  cmm. 
in  the  spinal  fluid?  Ordinarily  this  finding  would 
indicate  an  active  process.  Some  writers  have  claimed 
that  a  cell  count  running  above  100  per  cmm.  was  an 
indicator  of  diffuse  non-paretic  neurosyphilis.  It  does 
not  appear  that  this  claim  has  been  substantiated. 
It  is  remarkable  that  this  case  shows  an  interval  of  40 
years  between  infection  and  the  occurrence  of  definite 
clinical  symptoms.  With  respect  to  the  cell  count, 
both  in  untreated  and  in  treated  cases,  the  following 
conclusions  from  a  recent  article  (Solomon  and  Koefod)* 
are  in  point: 

1.  The  number  of  cells  found  in  the  fluid  of  un- 
treated cases  offers  no  definite  information  of  prog- 
nostic value. 

2.  One   is   not   justified   in   drawing   any   conclu- 
sions as  to  whether  the  case  is  cerebrospinal  syphilis 
or  general  paresis,  nor  the  time  the  process  has  been 
active,  nor  the  severity  of  it,  from  the  cell  count. 

3.  The  cell  count  may  vary  greatly  from  month  to 
month,  or  when  the  interval  is  but  several  days,  while 
at  other  times  it  may  remain  very  nearly  the  same 
after  an  interval  of  months. 

4.  Cases  showing  natural  remissions  may  show  no 
reduction  in  the  cell  count,  or  other  spinal  fluid  findings. 

*"The  Significance  of  Changes  in  Cellular  Content  of 
Cerebrospinal  Fluid  in  Neurosyphilis,"  Boston  Medical  and 
Surgical  Journal,  CLXXIII,  27. 


244  PUZZLES  AND   ERRORS 

5.  Cases   treated  with   salvarsan,    either   intraspi- 
nously  or  intravenously,  tend  to  show  a  more  or  less 
rapid  fall  in  the  cell  count.     This  count  will,  as  a  rule, 
remain  low  during  treatment,  but  is  likely  to  rise  when 
treatment  has  been  discontinued,  but  may  rise  during 
treatment  after  having  first  fallen. 

6.  Cases   may   show  remissions  during  treatment 
and  still  have  a  pleocytosis. 

7.  Treated  cases  having  the  cell  count  fall  to  normal 
may  at  the  same  time  become  very  much  worse  and 
develop  more  marked  paralytic  symptoms. 

8.  In  general  paresis  the  cell  count  in  no  way  paral- 
lels the  other  spinal  fluid  findings. 

-  9.  In  cases  in  which  the  other  tests  show  an  im- 
provement, for  instance  cerebrospinal  syphilis,  the  cell 
count  also  readily  and  early  drops  to  normal.  At  times 
it  may  drop  to  normal  before  other  spinal  fluid  tests 
become  negative;  again  it  may  be  last  to  reach  normal. 

10.  The   change   in   cell    count   seen   in   syphilitic 
disease  untreated  is  also  found  in  non-syphilitic  diseases, 
as  brain  tumor. 

11.  The    cell   count    offers   nothing  of    prognostic 
importance  in  syphilis  of  the  nervous  system  unless  ac- 
companied  by  improvement  of   the  other  laboratory 
signs. 

12.  The  cell  count  is  not  an  index  to  the  predomi- 
nance of  irritative  or  degenerative  changes. 


PUZZLES   AND   ERRORS  245 


Case  of  CEREBRAL  MALARIA  and  SYPHILIS: 
simulation  of  PARETIC  NEUROSYPHELIS  ("  gen- 
eral paresis  "). 


Case  67.  Joseph  Temple,  45,  who  had  been  a  sea-going 
steamboat  steward,  was  brought  to  the  hospital  in  a  semi- 
stupor.  He  was  entirely  uncooperative,  often  resistive,  at- 
tempting to  bite  the  physician's  fingers,  and  for  the  most 
part  lying  curled  up.  He  was  incontinent  and  tube-fed. 
This  phase,  it  seems,  had  begun  the  night  before  entrance  to 
the  hospital.  Twenty-four  hours  later,  an  extraordinary 
change  was  noted.  Temple  became  alert  and  attended  to  his 
wants,  began  to  eat  well,  and  began  to  behave  as  normally 
as  probably  he  ever  behaved. 

He  was  now  able  to  give  a  coherent  history.  It  was  now 
January.  In  the  previous  September,  he  had  left  for  Mexico; 
he  was  returning  when  he  suddenly  fell  to  the  deck,  uncon- 
scious. After  this  fall,  he  had  not  been  well,  having  had 
chills  and  fever.  At  the  Marine  Hospital,  he  had  been 
diagnosed  as  suffering  from  malaria,  and  was  given  quinin. 
He  had  been  delirious  a  short  time  in  the  hospital,  not  being 
able  to  recognize  his  wife,  who  called.  He  shortly  improved 
so  that  his  wife  was  able  to  take  him  home.  Nevertheless, 
headache,  gastric  distress,  and  intermittent  vomiting  con- 
tinued. A  spell  of  confusion  took  place,  two  days  before 
admission.  The  patient  tossed  about,  moaned,  and  failed 
to  recognize  anyone.  Malaria  of  the  sestivo-autumnal  type 
was  demonstrated  in  the  hospital.  The  temperature  always 
remained  at  normal.  He  was  somewhat  emaciated  and  pale. 
The  pupils  were  small,  somewhat  unequal,  and  reacted  though 
poorly  to  light  and  distance.  The  tendon  reflexes  were 
lively. 

The  W.  R.  of  the  serum  was  positive,  and  information  from 
the  patient's  physician  runs  to  the  effect  that  there  was  a 
syphilitic  infection  some  seven  or  eight  years  ago,  followed 
by  secondary  symptoms,  but  the  patient  had  refused  to  take 


246  PUZZLES  AND   ERRORS 

any   protracted   treatment.     The   spinal   fluid   examination 
was  practically  negative. 

Mentally,  the  patient  was  euphoric,  expansive,  boastful, 
and  showed  a  marked  emotional  instability  and  considerable 
memory  defect. 

I.  Can  the  diagnosis  of  general  paresis  be  made  in  Joseph 
Temple?  Certainly  the  acute  confusion  and  the  syncope 
are  consistent  enough  with  the  diagnosis,  yet  the  severe 
malaria  makes  it  seem  likely  that  the  phenomena  were 
due  to  a  cerebral  attack  of  malaria,  and  such  occur- 
rences are  found  in  the  sestivo-autumnal  form  of  ma- 
laria. Yet  malaria  would  hardly  explain  the  euphoria, 
memory  defect,  and  the  pupillary  findings,  to  say  noth- 
ing of  the  irritability  and  the  active  tendon  reflexes. 
Even  if  we  regard  the  active  tendon  reflexes  and  the 
irritability  as  malarial,  the  other  phenomena  remain 
outstanding  as  exceedingly  suspicious  of  paresis. 

On  the  other  hand,  if  we  try  to  support  forcibly  the 
diagnosis  of  general  paresis,  we  are  hardly  able  to  ex- 
plain the  negative  findings  in  the  spinal  fluid. 

In  point  of  fact,  a  study  of  the  patient's  past  life 
revealed  a  story  that  the  mental  traits  of  euphoria, 
irritability,  and  memory  defect  had  been  characteristic 
of  the  patient  for  many  years.  In  fact,  there  is  some 
question  whether  the  patient  is  not  really  to  be  regarded 
as  a  moron  of  high  grade. 

Upon  this  basis,  if  we  regard  the  confusional  phe- 
nomena as  malarial  and  the  persistent  mental  phenomena 
as  characteristic  of  a  moron  and  somewhat  exaggerated 
by  the  disease,  we  have  merely  to  explain  the  suggestive 
pupils.  As  to  these,  it  must  be  remembered  that  though 
they  reacted  poorly  to  light,  still  they  reacted  somewhat, 
so  it  is  not  a  question  of  explaining  an  Argyll- Robertson 
pupil,  but  only  an  impaired  pupillary  reaction.  Of 
course,  some  workers  are  of  the  opinion  that  pupil- 
lary changes,  perhaps  even  the  Argyll- Robertson  pupils, 
may  occur  in  syphilitic  cases  that  are  not  neurosyphilitic, 
or  at  all  events  are  not  victims  of  central  neurosyphilis. 
Finally,  we  must  remember  that  there  are  cases  of 
neurosyphilis  of  a  vascular  type  which  yield  negative 
spinal  fluids.  The  case  leaves  many  questions  unan- 
swered. 


PUZZLES   AND   ERRORS  247 


Can  paretic  and  non-paretic  neurosyphilis  be  dif- 
ferentiated by  means  of  the  gold  sol  reaction? 
The  gold  sol  reaction  in  this  case  was  an  extremely 
mild  one  and  would  not  at  all  have  warranted  the 
diagnosis  GENERAL  PARESIS,  yet  the  discovery 
of  a  heavy  meningeal  exudate  including  an  un- 
usually heavy  deposit  of  plasma  cells  even  in  the 
spinal  pia  mater  will  perhaps  warrant  us  in  making 
a  final  retrospective  diagnosis  of  paretic  neuro- 
syphilis. Autopsy. 


Case  68.  We  would  like  to  give  the  full  effect  of  our  sur- 
prise at  the  outcome  of  the  case  of  Margaret  O'Brien,  a 
school-teacher,  26  years  of  age.  To  be  sure,  Miss  O'Brien 
developed  symptoms  at  22  or  23  which  we  can  now  explain 
consistently  with  the  outcome  of  the  case;  for  at  that  time, 
she  began  to  complain  of  severe  pain  in  the  head,  especially 
in  the  forehead  and  temples,  and  also  became  nervous,  unable 
to  remain  quiet,  and  given  to  insomnia.  She  was  markedly 
depressed  at  the  time  and  would  refuse  to  talk  at  times. 
However,  only  the  headache  in  this  prodromal  period  could 
be  regarded  as  particularly  suggestive  of  syphilis,  and  head- 
ache in  an  over-worked  school-teacher  is  not  uncommon. 

In  fact,  the  picture  presented  by  the  patient  was  one  of 
catatonic  dementia  praecox.  The  patient  was  admitted  to 
the  hospital  after  a  sudden  onset  of  excitement.  At  first  she 
was  very  restless,  continually  looking  about  and  getting  up 
and  walking  away  from  the  examiner,  giving  the  impression  of 
understanding  all  questions  but  preserving  an  air  of  indif- 
ference. A  few  days  later,  the  patient  was  gotten  to  answer 
more  cooperatively.  She  remarked  that  the  hospital  was 
heaven  although  in  Boston;  that  it  was  summer  time  (correct) 
and  that  her  memory  was  greatly  impaired.  The  physician 
was  a  messenger  of  God  (delusion  later  corrected).  The 
patient  had  not  done  God's  will;  her  breath  was  leaving  her; 
God's  voice  was  heard  from  time  to  time,  and  Miss  O'Brien 


248  PUZZLES   AND   ERRORS 

had  heard  it  for  a  long  time.  God  tells  her  to  do  His  will. 
However,  as  Miss  O'Brien  remarked,  "  I  must  think  all  this 
nonsense,  turning  against  God." 

The  patient  frequently  attitudinized  and  would  remain 
in  an  apparently  catatonic  condition  for  many  minutes. 
For  the  most  part,  she  was  resistive  and  mute  and  non- 
cooperative  as  to  examination.  From  time  to  time,  she 
made  impulsive  suicidal  attempts.  So  far  as  a  somewhat 
inadequate  physical  examination  was  concerned,  nothing  ab- 
normal could  be  made  out;  in  particular,  the  pupils  reacted 
normally  to  light  and  were  otherwise  normal.  The  routine 
W.  R.  of  the  blood  serum,  however,  returned  positive,  and 
in  accordance  with  the  policy  of  the  Psychopathic  Hospital, 
the  patient  was  subjected  to  a  lumbar  puncture.  The  lum- 
bar puncture  yielded  a  positive  W.  R.,  109  cells  per  cmm.,  a 
positive  globulin  and  a  considerable  excess  of  albumin,  and 
an  exceedingly  mild  gold  reaction  —  syphilitic  type. 

Ten  days  after  admission,  the  patient  had  a  convulsion. 
She  never  regained  consciousness,  continued  to  have  convul- 
sions for  a  few  hours,  and  died,  apparently  from  paralysis 
of  respiration.  The  heart  continued  to  beat  for  a  short 
period  after  respiration  ceased.  The  autopsy  was  consis- 
tent with  the  diagnosis  which  had  been  rendered  after  the 
surprising  results  of  the  W.  R.  in  the  blood  and  the  laboratory 
findings  in  the  spinal  fluid  had  been  learned.  There  was  a 
generalized  encephalitis  with  congestion  of  all  the  smaller 
cerebral  vessels  and  petechial  areas  in  the  meninges  and 
upon  the  cortical  surfaces.  We  regard  the  case  as  one  of 
syphilitic  encephalitis. 

The  brain  weighed  1265  grams,  indicating  a  loss  of  79 
grams  by  Tigges'  formula  (8  times  the  body  length  in  centi- 
metres). The  pia  mater  was,  in  the  gross,  quite  normal 
within  the  cranium;  nor  were  any  cells  found  in  a  smear 
from  this  pia  mater;  but  the  pia  mater  over  the  spinal  cord 
was  visibly  edematous,  and  a  smear  from  the  spinal  pia 
mater  showed  great  numbers  of  lymphocytes  and  especially 
of  plasma  cells  —  a  finding  which  was  confirmed  in  stained 
section,  by  which  a  remarkable  display  of  plasma  cells  was 
found  plastered  somewhat  generally  over  the  entire  pia 


PUZZLES   AND   ERRORS  249 

mater  of  certain  segments.  The  brain  substance  was  softer 
than  normal,  but  displayed  no  differences  of  consistence. 
The  stripping  of  the  pia  mater  of  the  temporal  lobes  on  both 
sides  yielded  the  so-called  "  decortication  "  (that  is,  the  adhe- 
sion of  small  bits  of  brain  substance  to  the  pia  mater).  The 
optic  nerves  were  somewhat  thinner  than  normal.  No  other 
gross  lesions  of  the  brain  were  found. 

The  dura  mater,  although  dense  and  injected,  was  not 
otherwise  abnormal.  There  was  an  early  visible  sclerosis 
of  the  middle  meningeal  arteries,  more  marked  on  the  left 
side. 

The  cause  of  death,  so  far  as  the  autopsy  revealed  it, 
was  bronchial  pneumonia.  There  was  a  diffuse  nephritis. 

1.  Are  the  hallucinations  in  the  case  of  O'Brien  character- 

istic? Hallucinations  are  regarded  as  playing  a  minor 
r61e  in  general  paresis.  In  fact,  earlier  workers  some- 
times denied  that  hallucinations  occurred  at  all,  and 
this  denial  has  been  made  once  more  of  late  by  Plaut,* 
but  Kraepelin  quotes  Obersteiner  as  observing  hallu- 
cinations in  10%,  and  regards  that  figure  as  approx- 
imately corresponding  with  his  own  experience.  Junius 
and  Arndt  are  cited  as  finding  17%  of  their  cases  hallu- 
cinated. Auditory  hallucinations  are  somewhat  more 
frequent  than  those  of  vision  (alcoholic  psychosis  must 
be  considered) .  The  visual  hallucinations  of  paresis  are 
thought  by  Kraepelin  to  be  related  with  atrophy  of  the 
optic  nerves,  and  he  states  that  they  occur  by  prefer- 
ence in  patients  having  such  atrophy.  Hallucinations 
though  not  common  are  more  frequent  in  non-paretic 
neurosyphilis  than  in  paretic  neurosyphilis. 

2.  What   was   the   cause   of   death   in   Margaret   O'Brien? 

The  autopsy,  as  above  stated,  indicated  pneumonia. 
In  point  of  fact,  this  patient  developed  convulsions  and 
ceased  respiration,  the  heart  continuing  to  beat  for 
some  time  after  respiration  had  ceased.  It  may  be 
that  the  death  should  be  counted  as  one  of  neuro- 
syphilitic  seizure. 

*  Plaut:  Ueber  Halluzinosen  der  Syphilitiker,  Berlin,  1913. 


250 


PUZZLES  AND   ERRORS 


Tonsillar    abscess 
(Lues  Maligna?). 

associated   with    neurosyphilis 

Case  69.  Frank  Mason,  49  years,  a  rectifier  of  spirits, 
was  admitted  to  the  Psychopathic  Hospital  in  a  tremulous, 
mentally  confused,  depressed,  and  unhappy  state.  He  was 
particularly  concerned  because  he  could  not  give  an  accurate 
account  of  his  past  life  and  because  he  found  that  he  was 
continually  contradicting  himself. 

Superficial  examination  shortly  discovered  the  pupils  to  be 
much  contracted,  irregular,  and  non-reactive  either  to  light 
or  distance.  Although  these  pupils  showed  more  than  the 
Argyll- Robertson  phenomenon,  yet  the  suspicion  of  syphilis 
was  important. 

Throat  examination  showed  a  large  area  of  ulceration 
involving  the  whole  of  the  right  tonsil  and  extending  even 
to  the  left  side  of  the  median  line  so  that  the  whole  of  the 
faucial  pillar  was  involved.  In  the  midst  of  this  ulcerative 
area  was  a  mass  of  purulent  necrotic  tissue,  about  which  the 
edges  of  the  ulcer  stood  out  sharply.  There  was,  however, 
very  little  acute  reaction  about  the  margin  of  the  area. 

The  association  of  pupillary  changes  (especially  stiffness 
to  light),  what  looked  like  tonsillar  gumma,  and  mental  dis- 
order (including  memory  disturbance)  heightened  the  impres- 
sion of  syphilis. 

However,  the  remainder  of  the  examination  was  not  es- 
pecially confirmatory  of  the  diagnosis.  The  man  was  well 
developed  and  obese,  with  a  slightly  enlarged  heart,  with 
sounds  of  poor  quality  and  the  aortic  second  sound  accen- 
tuated. The  systolic  blood  pressure  was  130;  the  diastolic, 
90.  There  was  no  disorder  of  reflexes  except  that  the  arm 
reflexes  were  very  lively. 

After  a  time,  a  few  facts  concerning  the  patient's  life 
became  available.  Although  a  rectifier  of  spirits,  Mason 
could  not  be  found  to  have  over-indulged  in  alcohol.  It 
appears  that  some  five  months  before  his  admission  to  the 


PUZZLES   AND   ERRORS  2$I 

hospital,  a  wisdom-tooth  had  been  extracted.  About  four 
months  before  admission,  the  ulceration  of  the  faucial  pillar 
had  begun,  and  this  ulceration  was  immediately  laid  to  in- 
fection from  the  wisdom-tooth  cavity.  Mason  then  had  to 
discontinue  work  and  a  depression  followed.  But  the  account 
of  this  depression  led  us  to  think  that  he  was  a  victim  more 
of  natural  sadness  than  psychopathic  depression.  There 
was  much  worry  and  insomnia.  To  meet  the  insomnia, 
large  amounts  of  hypnotics  were  administered.  The  sequence 
of  these  hypnotics  was  a  tremendous  disturbance  and  contin- 
ual crying  out  by  the  patient.  In  fact,  Mason  became  so 
excited  that  he  was  removed  to  the  Psychopathic  Hospital 
for  temporary  care  in  the  condition  above  mentioned. 

We  naturally  awaited  the  outcome  of  the  serum  W.  R. 
The  return  was  negative.  However,  the  typical  position  of 
the  ulcerative  lesion  and  the  non-reacting  pupils,  —  to  say 
nothing  of  the  mental  symptoms  and  the  associated  tremors, 
with  incoordination  (this  incoordination  was  non-characteris- 
tic and  apparently  due  largely  to  the  tremor),  —  led  to  lumbar 
puncture. 

The  spinal  fluid  yielded  a  weakly  positive  W.  R.  There 
was  a  slight  positive  albumin,  the  globulin  test  was  slightly 
positive,  there  were  14  cells  per  cmm.,  and  the  gold  sol  reac- 
tion was  of  the  syphilitic  type.  We  were,  then,  probably 
entitled  to  conclude  that  syphilis  was  active  not  only  in  the 
body  at  large  but  also  in  the  nervous  system.  Looking  back 
upon  the  case,  we  considered  that  large  doses  of  morphine  and 
hyoscyamus  might  well  have  produced  the  marked  mental 
confusion  and  possibly  the  tremors  that  characterized  Mason 
on  his  arrival  at  the  hospital. 

Improvement  followed  after  a  few  days  of  rest;  the  con- 
fusion disappeared  and  the  tremors  diminished;  the  pupils 
returned  to  their  normal  size  and  reaction;  depression  per- 
sisted, and  the  patient  was  very  properly  much  concerned 
about  the  tonsillar  lesion.  However,  further  improvement 
did  not  take  place  under  antisyphilitic  treatment  and  patient 
died  after  several  weeks  from  what  was  believed  to  be  an 
embolus  from  the  tonsil. 


252  PUZZLES  AND   ERRORS 

1.  What  was  the  true  interpretation  of   Frank  Mason's 

pupillary  changes?  They  were  probably  due  to  the 
opiates,  despite  the  fact  that,  taken  in  association  with 
the  gummatous  lesion  of  the  faucial  pillar,  we  had  re- 
garded them  as  possibly  syphilitic. 

2.  How  shall    the  negative  serum  W.   R.   be    explained? 

Such  a  reaction  is  consistent  with  the  diagnosis  gumma. 
It  is,  however,  a  little  surprising  that  with  active  neuro- 
syphilis  and  a  relatively  active  non-nervous  syphilitic 
lesion  like  that  in  this  case,  the  serum  W.  R.  should 
have  been  negative.  Possibly  a  repetition  of  the  test 
at  various  times  would  have  shown  a  positive  serum 
W.  R.  In  any  event,  the  fluid  reaction  was  positive. 

3.  Could  the  tonsillar  ulceration  be  due  to  dental  infection? 

The  chances  are  against  this  on  account  of  the  interval 
(2  months)  between  extraction  of  the  wisdom  tooth 
and  the  ulceration,  which  itself  seems  to  be  of  a  ter- 
tiary syphilitic  nature.  In  point  of  fact,  the  patient 
admitted  a  syphilitic  infection  21  years  previously 
namely,  at  28  years  of  age.  At  that  time  he  took 
large  quantities  of  mercury  and  potassium  iodid  by 
mouth. 

4.  Relation  of  the  case  of  Frank  Mason  to  the  so-called 

lues  maligna?  The  case  closely  resembled  the  cases 
reported  by  Ely.  Frank  Mason  showed  great  destruc- 
tion of  tissue,  toxemia,  failure  to  react  to  antisyphilitic 
treatment.  In  both  of  Ely's  cases,  the  tonsil  was  the 
starting  point  of  the  illness ;  and  in  both  cases  there  was 
a  trauma  of  the  tonsil  or  peri-tonsillar  structures  (ton- 
sillectomy  and  application  of  caustic).  In  our  case 
there  not  only  had  been  extraction  of  a  wisdom  tooth, 
but  the  tonsil  had  been  cauterized. 


PUZZLES  AND  ERRORS  253 


Neurosyphilis  versus  multiple  sclerosis. 


Case  70.  Annie  Kelly  is  a  young  Irish  woman,  21  years  of 
age,  who  was  perfectly  well  until  three  months  before  her 
admission  to  the  Psychopathic  Hospital,  when  suddenly 
one  evening  she  became  very  dizzy.  This  was  followed  by  a 
chill  and  vomiting.  The  next  day  she  had  a  sore  throat  but 
was  able  to  be  about  and  do  her  work.  The  dizziness, 
however,  continued  and  she  began  to  feel  rather  queer. 
Gradually  it  became  difficult  for  her  to  walk  on  account  of 
staggering. 

A  little  later  she  noticed  a  weakness  of  the  left  side,  in- 
volving face,  arm,  and  leg;  then  she  began  to  find  it  diffi- 
cult to  talk.  Finally  the  right  leg  became  weak,  making 
walking  practically  impossible.  All  these  symptoms  grew 
worse  and  the  dizziness  increased.  At  times  her  vision  would 
be  blurred ;  there  were  somewhat  frequent  attacks  of  diplopia. 
Finally  she  had  to  take  to  her  bed,  and  at  last  she  lost  con- 
trol of  her  sphincters. 

At  no  time  did  she  suffer  any  pain.  She  was  taken  to  a 
hospital,  and  after  a  time  improved  somewhat;  but  she  was 
told  she  had  a  brain  tumor  and  had  better  be  in  a  large  city, 
where  she  could  have  surgical  aid  if  this  became  necessary; 
consequently,  she  was  brought  from  Montana  to  Boston. 

On  admission  to  the  hospital,  the  examination  disclosed  no 
important  symptoms  outside  of  the  nervous  and  locomotor 
systems.  She  was  unable  to  walk  unless  assisted.  The 
pupils  were  large  but  reacted  well  to  both  light  and  accommo- 
dation, were  equal  in  size,  and  regular.  Slight  nystagmus 
was  present;  there  was  no  ptosis  or  strabismus;  vision  in 
the  left  eye  was  poor.  The  other  cranial  nerves  showed 
no  involvement.  The  tendon  reflexes  were  all  present  and 
very  lively;  Babinski,  Gordon,  and  Oppenheim  signs  were 
present  on  either  side.  The  ataxia  was  marked,  especially 
of  the  lower  arms,  and  she  had  some  difficulty  in  the  align- 
ment of  the  fingers.  The  sense  of  position  of  the  limbs  was 


254  PUZZLES  AND   ERRORS 

very  poor.  There  was  some  tremor,  which  was  not  of  the 
intention  type.  The  writing  showed  some  incoordination. 
The  speech  showed  nothing  abnormal.  Mental  examination 
disclosed  nothing  of  note  objectively,  but  patient  stated  she 
could  not  think  so  clearly  as  she  could  formerly. 

The  diagnosis  would  seem  to  lie  between  brain  tumor,  — 
which  had  been  suggested  to  the  patient  by  her  physician,  — 
multiple  sclerosis,  and  neurosyphilis.  The  numerous  neuro- 
logical symptoms  without  any  definite  evidence  of  intra- 
cranial  pressure  were  sufficient  to  rule  out  for  the  moment 
the  consideration  of  brain  tumor.  The  syndrome  of  multiple 
sclerosis  is  not  complete,  but  the  race,  age,  and  onset,  with 
the  increasing  and  decreasing  intensity  of  symptoms  are 
very  suggestive  of  this  diagnosis.  The  symptoms,  of  course, 
are  all  consistent  with  neurosyphilis  also,  and  while  the  pa- 
tient denied  any  knowledge  of  syphilitic  involvement,  the 
examination  of  the  blood  and  spinal  fluid  was  made.  The 
W.  R.  was  negative  in  both  the  blood  serum  and  spinal  fluid. 
Further  examination  of  the  spinal  fluid  showed  presence  of 
globulin  and  an  increase  in  the  albumin  content,  43  cells 
per  cmm.  and  a  "  paretic  "  type  of  gold  sol  reaction.  With 
the  negative  W.  R.  of  both  blood  serum  and  spinal  fluid, 
and  with  so  much  in  favor  of  MULTIPLE  SCLEROSIS,  this 
diagnosis  was  made. 

1.  What  is  the  relation  of  multiple  sclerosis  to  syphilis? 

There  is  no  definite  relationship  between  multiple 
sclerosis  and  syphilis,  —  that  is,  multiple  sclerosis  is 
not  a  syphilitic  disease;  but  the  complete  syndrome 
of  multiple  sclerosis  is  often  given  by  a  syphilitic  in- 
volvement of  the  central  nervous  system  (see  case 
Lauder,  71). 

2.  Is  the  spinal  fluid  finding  in  this  case  consistent  with 

multiple  sclerosis?  According  to  Nonne,  about  19% 
of  the  cases  of  multiple  sclerosis  show  globulin  and 
pleocytosis  in  the  spinal  fluid.  As  a  rule,  the  number  of 
cells  ranges  between  10  and  20  per  cmm.  and  the  glob- 
ulin is  not  present  in  large  amounts.  In  this  case,  the 
amount  of  globulin,  which  was  given  as  2  +  ,  is  only  a 
moderate  amount,  —  less  than  is  usually  found  in 
cases  of  general  paresis.  There  are  not  very  many 


PUZZLES   AND   ERRORS  255 

cases  of  multiple  sclerosis  in  the  literature  in  which 
a  gold  sol  reaction  has  been  performed,  but  in  the 
majority  of  those  tested,  the  reaction  is  reported  as 
mild.  However,  cases  of  multiple  sclerosis  giving  a 
typical  paretic  curve  have  been  described  by  a  number 
of  observers,  among  whom  may  be  mentioned  Kaplan 
and  Solomon. 

How  frequently  is  it  necessary  to  make  a  differential 
diagnosis  between  multiple  sclerosis  and  neurosyphilis? 
Before  the  days  of  the  W.  R.  this  differentiation  was 
much  more  difficult  than  at  present.  But  we,  however, 
still  have  to  face  a  not  very  rare  difficulty  in  separating 
the  two  conditions.  Syphilis  is  prone  to  cause  small 
localized  lesions  in  the  nervous  system.  The  changes 
in  the  patient's  condition,  with  improvements  and 
regressions  are  equally  characteristic  of  both  diseases. 
How  closely  the  symptomatology  of  neurosyphilis  may 
simulate  that  of  typical  multiple  sclerosis  is  shown  in 
the  next  case  (Lauder,  71).  When  the  sclerotic  area 
of  multiple  sclerosis  occurs  in  appropriate  parts  of 
the  cerebrum,  symptoms  of  mental  disturbances  will 
occur.  In  its  histological  picture  multiple  sclerosis  is 
at  times  highly  suggestive  of  syphilis,  even  showing 
mononucleosis  and  meningitis. 


256  PUZZLES  AND   ERRORS 


Optic  atrophy;   nystagmus;    spasticity;    intention 
tremor.    Diagnosis :    ? 


Case  71.  James  Lauder  began  to  lose  his  eyesight  at  32 
years,  and  was  shortly  determined  to  be  suffering  from 
primary  optic  atrophy.  In  the  course  of  a  year,  he  had 
become  completely  blind.  No  mental  symptoms  had  de- 
veloped. 

Physically,  Lauder  was  in  very  good  condition.  Neuro- 
logically,  there  was  a  complete  optic  atrophy  with  paralysis 
of  the  internal  rectus  muscle,  marked  nystagmus,  and  ab- 
sent pupillary  reactions.  All  the  tendon  reflexes  were  ex- 
ceedingly lively,  though  the  right  arm  reflexes  were  more 
lively  than  the  left,  and  the  left  leg  reflexes  more  lively  than 
the  right.  There  was  an  ankle  clonus  on  both  sides.  The 
abdominal  and  cremasteric  reflexes  were  lively.  There  was 
a  slight  intention  tremor.  There  was,  however,  no  ataxia 
and  no  speech  defect. 

Diagnosis:  The  nystagmus,  optic  atrophy,  and  the  re- 
flex disorder  suggested  multiple  sclerosis,  although  the 
liveliness  of  the  superficial  reflexes,  especially  the  abdominal 
reflexes,  was  a  point  somewhat  against  any  advanced  degree 
of  multiple  sclerosis.  It  would  appear  that  the  absence  of 
pupillary  reaction  to  accommodation  is  also  rather  unusual 
in  multiple  sclerosis. 

The  serum  and  spinal  fluid  W.  R.'s  proved  positive.  There 
were  25  cells  per  cmm.,  albumin  was  in  excess,  and  there  was 
a  positive  globulin  reaction. 

i.  What  is  the  significance  of  optic  atrophy  and  other 
optic  changes  with  respect  to  neurosyphilis?  Canavan, 
from  our  laboratory,  has  reported  that  she  found  that 
40  of  58  unselected  cases  of  mental  disease  exhibited 
obvious  and  undeniably  important  changes  in  the 
optic  nerve.  She  found  that  optic  nerve  changes  were 
even  more  frequent  than  chronic  spinal  cord  changes 
as  detectable  by  the  same  method  (Weigert  myelin 


PUZZLES  AND   ERRORS  257 

sheath  method);  there  were  only  34  of  the  58  cases 
which  showed  chronic  spinal  cord  changes.  Eighteen 
cases  very  probably  syphilitic  (although  the  clinical 
evidence  was  not  in  all  cases  supported  by  the  W.  R.) 
failed  to  show  optic  nerve  changes  in  but  three  in- 
stances. The  15  syphilitic  cases  that  did  show  optic 
nerve  changes  showed  them  in  but  one  eye  in  three  cases, 
in  both  eyes  in  12  cases.  Canavan  incidentally  dem- 
onstrated a  spirochetosis  in  the  pial  sheath  of  the  optic 
nerve  in  a  case  of  neurosyphilis,  possibly  paretic. 
2.  What  is  the  frequency  of  eye  changes  in  neurosyphilis? 
Posey  and  Spiller  ("  The  Eye  and  the  Nervous  System," 
1906)  quote  Keraval  as  finding  42  instances  of  fundus 
change  in  51  cases  of  paresis.  Clifford  Allbutt  found 
41  cases  of  atrophy  in  53  of  paresis;  other  authors  have 
found  far  fewer.  Optic  atrophy  sufficiently  marked  to 
cause  blindness  is  relatively  rare  in  paresis.  Com- 
pare table  of  eye  changes  from  Joffroy  under  Case 
Falvey(55). 

As  for  optic  atrophy  in  tabes,  Posey  and  Spiller 
record  statistics  as  so  various  as  to  be  on  the  whole 
unsatisfactory.  The  highest  percentages  found  appear 
to  be  those  of  Mott,  80%,  and  Gross,  88%.  It  is  evi- 
dent that  the  standards  for  measuring  optic  atrophy 
must  differ  very  much. 


258  PUZZLES   AND   ERRORS 


Atypical  case  of  neurosyphilis.    Picture  of  Hunt- 
ington's  chorea. 


Case  72.  Margaret  Green,  28,  was  received  at  Danvers 
State  Hospital  in  an  excited  and  frightened  state.  She  was 
very  talkative  and  said  that  she  was  being  bitten  by  snakes 
and  serpents.  She  thought  every  one  approaching  her  was 
the  devil,  and  sprinkled  what  she  called  "  holy  water  "  about 
her  for  protection.  It  was  clear  that  she  was  hallucinated. 
She  heard  her  child  crying,  and  she  saw  a  woman  carrying  it 
away. 

After  a  few  weeks,  Mrs.  Green  grew  quiet  and  more  rational 
except  for  a  few  spells  of  violence  and  noise;  she  gave  the 
impression  of  a  rather  pleasant  and  agreeable,  though  some- 
what demented,  patient.  Physically,  beyond  a  tremor  of 
fingers  and  tongue  and  lively  knee-jerks  and  some  evidence 
of  enlargement  of  the  heart,  there  was  nothing  to  be  found. 

Margaret  Green  is  still  in  the  Danvers  Hospital,  being  now 
48  years  of  age.  During  the  twenty  years,  she  has  presented, 
-  besides  the  mental  picture  of  impairment  of  memory  — 
occasional  spells  of  confusion,  a  variety  of  delusions  based, 
at  least  in  part,  upon  auditory  and  vivid  visual  hallucinations, 
a  certain  irritability  and  psychomotor  excitement,  and  a 
picture  of  Huntington's  chorea.  The  diagnosis  of  Hunting- 
ton's  chorea  has  always  been  in  doubt  by  reason  of  the  lack 
of  any  evidences  of  hereditary  taint;  it  has,  however,  not 
been  possible  to  secure  a  properly  intensive  account  of  her 
relatives. 

It  appears  that  the  choreic  movements  were  first  ob- 
served—  in  the  hospital  at  least  —  about  16  years  ago.  The 
patient  has  always  been  decidedly  mixed  upon  dates.  From 
internal  evidence  derived  from  her  obviously  in  part  erroneous 
statements,  it  may  be  that  the  chorea  began  at  the  age  of 
23.  It  appears  that  she  had  been  often  termed  a  victim  of 
of  St.  Vitus'  dance,  and  had  had  to  leave  her  work  in  the  mill 
on  account  of  the  disease.  From  one  source  of  information, 


PUZZLES   AND   ERRORS  259 

it  would  appear  that  the  patient  began  to  have  what  was 
called  St.  Vitus'  dance  when  she  was  14  or  15  years  of  age; 
so  far  as  this  informant  knew,  no  other  member  of  the  family 
had  had  the  affliction. 

The  first  movements  observed  in  the  hospital  were  irregular, 
jerking  movements,  more  marked  in  the  left  arm  but  also 
occurring  in  the  other  extremities,  as  well  as  in  the  face, 
wherein  were  produced  peculiar  grimaces.  The  twitching 
movements  would  become  decidedly  worse  during  spells  of 
irritability.  Observation  in  the  patient's  early  thirties  left 
the  question  in  doubt  whether  the  left  pupil  reacted  to 
light  or  not.  In  1904,  when  the  patient  was  36,  both  pupils 
failed  to  react  to  light  either  directly  or  consensually.  At 
this  time,  the  jerky  movements  continued,  especially  in  the 
left  hand  and  forearm,  the  tongue  was  tremulous,  test 
phrases  were  poorly  pronounced,  the  knee-jerks  were  exag- 
gerated (especially  the  left),  and  both  wrist-jerks  were  exag- 
gerated. The  systematic  examination,  however,  revealed 
no  other  neurological  disorder.  Within  a  year,  slight  spuri- 
ous ankle  clonus  developed  on  both  sides;  the  eyes,  es- 
pecially the  left,  gave  the  appearance  of  developing  cata- 
racts. A  slight  consensual  light  reaction  was  demonstrable 
on  the  right  side,  but  all  light  reactions  were  absent  in  the 
left  eye. 

At  the  age  of  42,  the  patient  was  still  disoriented  for  time, 
place,  and  persons  and  subject  to  a  deep  amnesia;  was 
tidy,  tranquil,  and  of  a  pleasant  demeanor,  but  many  of  her 
muscles  were  in  continual  motion.  There  were  chewing 
movements  and  both  hands  and  feet  were  rarely  still.  There 
were  no  longer  any  spells  of  irritability  or  violence;  and 
once  when  found  crying  on  the  piazza,  Mrs.  Green,  on  being 
asked  the  reason,  replied  that  a  gray  cat  had  come  and  looked 
at  her  so  hard  it  made  her  cry.  There  were  other  crying 
spells  at  times  for  equally  good  reasons,  or  for  no  reason. 

More  recently,  the  patient  has  become  fairly  well  oriented 
for  time  and  place,  and  has  acquired  a  fairly  good  insight 
into  her  condition  and  a  good  memory  for  past  events. 
She  has  had  occasionally  auditory  hallucinations,  as  of  water 
running.  In  1914,  it  was  reported  that  the  pupils  reacted 


26O  PUZZLES  AND   ERRORS 

to  light,  and  the  rest  of  the  systematic  neurological  examina- 
tion was  negative  except  that  the  knee-jerks  were  exaggerated ; 
and  a  re-examination  in  1916  showed  the  pupils  still  reacted  to 
light.  At  present,  the  patient  is  disoriented  for  time,  stating 
that  her  age  is  about  25;  she  is  no  longer  subject  to  auditory 
hallucinations;  she  has  a  marked  difficulty  in  enunciation, 
emphasized  by  the  lack  of  teeth  and  in  part  due  to  continual 
movements  of  the  tongue ;  the  movements  appear  to  be  part 
of  a  generalized  chorea. 

In  a  systematic  review  of  the  Wassermann  findings  in  the 
hospital  population,  the  blood  of  Margaret  Green  was  ex- 
amined and  found  to  be  positive.  Lumbar  puncture  forth- 
with performed  showed  a  positive  W.  R.  in  the  fluid;  there 
was  a  positive  globulin  and  an  excess  of  albumin;  the  gold 
sol  was  characteristic  of  paresis;  there  were,  however,  but 
three  cells  per  cmm. 

I.  Are  the  choreiform  movements  related  to  the  demon- 
strable syphilis  of  the  nervous  system?  Neither  the 
fluid  W.  R.  nor  the  gold  sol  reaction  should  be  regarded 
as  necessarily  an  indicator  of  tissue  loss.  The  fluid 
W.  R.  is  commonly  thought  to  signify  merely  that  the 
fluid  contains  substances  which  are  somehow  due  to  the 
presence  of  spirochetes  in  some  region  pretty  closely 
related  with  the  fluid.  The  gold  sol  reaction,  although 
well  established  to  be  characteristic  of  neurosyphilis, 
is  perhaps  not  so  strong  an  evidence  of  the  existence  of 
spirochetes  in  the  region  from  which  fluid  constituents 
are  derived.  There  is  no  pleocytosis.  However,  the 
positive  globulin  test  and  the  excess  of  albumin  do 
indicate  a  certain  amount  of  destructive  process  some- 
where in  the  neural  tissues.  Are  we  to  suppose  that 
these  substances  have  been  continually  found  during 
the  course  of  this  disease?  This  question  cannot  be 
answered  with  the  data  in  hand,  and  we  can  only  sus- 
pect that  these  positive  tests  for  albumin  and  globulin 
are  an  effect  of  tissue  destruction  caused  by  neuro- 
syphilis. It  must  be  admitted  that  the  argument 
here  is  a  little  tenuous.  The  lesson  is  plain:  that 
in  the  present  stage  of  our  knowledge  the  W.  R. 
should  not  be  omitted  even  in  cases  which  present  a 
fairly  convincing  picture  of  some  well-known  entity. 
Thus,  a  disease,  which  looks  like  Huntington's  chorea, 


PUZZLES  AND  ERRORS  26l 

as  well  as  a  disease  suggestive  of  multiple  sclerosis, 
requires  investigation  by  the  methods  of  the  syphi- 
lographer. 

How  shall  we  explain  the  changes  in  pupillary  reaction 
in  this  case?  They  cannot  yet  be  explained.  A  few 
observers  have  reported  changes  in  pupillary  reflexes 
in  the  direction  of  normality.  In  our  experience  such 
changes  have  not  been  noted.  It  cannot  be  too  strongly 
emphasized  that  it  is  very  easy  to  make  errors  in 
judging  pupillary  reaction  if  care  is  not  used.  For 
instance,  if  the  patient  is  accommodating  for  near 
vision,  light  will  probably  not  cause  contraction.  A 
frequent  cause  of  error  in  testing  the  light  reflex  arises 
from  using  a  weak  electric  light.  An  electric  flash- 
light is  much  less  efficient  than  daylight.  Probably 
the  most  satisfactory  method  is  to  take  the  patient  to 
a  window,  ask  him  to  look  at  a  distant  object,  shade 
the  eye  with  the  hand,  remove  hand,  and  observe. 

What  is  the  chief  triad  of  symptoms  in  Huntington's 
chorea?  (i)  Choreiform  movements  associated  with 
(2)  progressive  mental  enfeeblement,  (3)  occurring  in 
a  patient  whose  family  history  shows  a  similar  con- 
dition in  a  preceding  generation. 


262  PUZZLES   AND   ERRORS 


Differential  diagnosis  between  NEURO SYPHILIS 
and  SENILE  ARTERIO SCLEROTIC  PSYCHOSIS. 


Case  73.  Marcus  Chatterton  was  a  retired  sea  captain, 
75  years  of  age.  At  the  age  of  71,  he  had  had  a  seizure  with 
a  slight  right  hemiplegia  and  inability  to  talk.  He  had  been 
slightly  confused  for  a  short  time  but  had  rapidly  recovered. 
During  the  intervening  four  years,  there  had  been  three 
similar  attacks,  and  the  last  one  had  caused  him  to  come  to 
the  hospital.  He  was,  in  fact,  confused  upon  admission 
but  had  become  perfectly  clear  by  the  next  day.  There  was 
a  considerable  memory  defect,  which  the  patient  himself 
did  not  entirely  appreciate.  Possibly  his  judgment  had  been 
deteriorating  slightly.  He  had  been  irritable  of  late  and 
sometimes  sleepless. 

Physical  examination  showed  a  rather  well-preserved 
man  with  but  slight  senile  changes.  The  pupils  were  equal 
and  reacted  readily  to  light  and  accommodation.  There 
was  no  sensory  disorder  and  no  disturbance  of  coordination. 
There  were  no  tremors.  The  systolic  blood  pressure  was  205, 
the  diastolic  135.  The  arteries  were  sclerotic  upon  palpation. 
A  sufficient  diagnosis  would  have  seemed  to  be  arteriosclerosis, 
and  the  hypothesis  of  syphilis  would  hardly  have  been  raised 
off-hand  by  most  practitioners.  The  W.  R.  of  the  serum  was 
negative.  What  led  to  lumbar  puncture  in  this  case  was  the 
fact  that  the  sea  captain's  wife  had  died  15  years  before  of 
general  paresis.  The  lumbar  puncture  was  rewarding  since 
the  W.  R.  was  positive.  There  was  an  increase  of  albumin 
and  globulin,  a  "  paretic  "  type  of  gold  sol  reaction,  and  56 
cells  per  cmm. 

Accordingly,  we  must  regard  the  condition  as  one  of 
neurosyphilis.  Perhaps  the  arteriosclerosis  was  of  syphilitic 
origin.  If  this  is  a  case  of  general  paresis  as  we  suppose, 
it  is  one  of  very  long-standing  syphilis. 


PUZZLES   AND   ERRORS  263 

1.  Do  delusions  of  grandeur  in  the  senile  period  suggest 

syphilis?  Not  necessarily;  it  appears  that  there  is  a 
small  group  of  senile  cases  which  might  be  called  cases 
of  senile  pseudoparesis  in  which  extravagant  delusions 
of  grandeur  are  entertained,  and  in  which  frontal 
atrophy  is  found  although  entirely  without  evidence  of 
chronic  inflammation.  It  has  not  been  proved  that 
these  cases  are  of  syphilitic  origin.  It  is  suggestive 
that  the  site  of  the  most  extensive  lesion  is  precisely 
the  site  of  the  most  extensive  lesion  classically  found  in 
paretic  neurosyphilis,  viz.,  in  the  frontal  regions. 

2.  Is  neurosyphilis   frequently   found  in  both   mates?     It 

can  hardly  be  said  that  this  is  a  usual  finding.  How- 
ever, it  is  far  from  rare,  and  it  occurs  frequently  enough 
to  be  used  in  support  of  the  theory  that  there  is  a 
special  strain  of  spirochete  that  has  a  predilection  for 
nervous  tissue.  It  must  be  remembered,  however, 
that  the  wives  of  syphilitics  are  frequently  infected 
without  being  aware  of  it.  In  such  cases  they  re- 
ceive no  treatment  and  consequently  have  a  larger 
chance  of  developing  neurosyphilis.  It  is  a  good  rule 
to  consider  the  mate  of  every  syphilitic  a  candidate  for 
neurosyphilis^ 


264  PUZZLES  AND   ERRORS 


An  atypical  case  of  recurrent  dazed  states  resem- 
bling HYSTERICAL  FUGUES.  Probably  an  in- 
stance of  NEUROSYPHILIS. 


Case  74.  Abel  Bachmann,  a  man  of  40  years,  remains 
doubtful  and  perhaps  belongs  to  the  still  unresolved  group 
of  mental  cases  due  to  syphilis  that  cannot  be  placed  in  any 
of  the  well-known  categories.  Bachmann  had  been  found  by 
the  police,  working  in  front  of  a  cowbarn  without  the  consent 
or  even  the  knowledge  of  the  owner.  Bachmann  had,  in 
fact,  spent  the  night  in  the  cowbarn  and  was  working  with 
the  idea  of  paying  for  his  night's  lodgings.  The  situation 
struck  the  police  as  so  peculiar,  and  Bachmann  was  so  co^ 
fused  and  irresponsive,  that  he  was  brought  to  the  Psycho- 
pathic Hospital.  The  afternoon  of  his  admission,  however, 
he  entirely  cleared  up  and  was  able  to  give  a  good  account  of 
himself. 

His  story  was  that  he  had  been  worrying  a  good  deal  about 
a  divorce  suit,  and  the  morning  of  his  episode  he  had  awakened 
with  peculiar  feelings.  He  walked  from  Boston  to  Cambridge, 
feeling  that  he  was  in  a  strange  city.  He  recognized  the 
places  he  passed,  yet  they  all  seemed  to  be  changed.  Upon 
reaching  Harvard  Square,  he  determined  to  return  to  Boston 
and  walked  and  walked,  failing  to  reach  Boston.  All  day  he 
had  eaten  nothing;  when  night  fell  he  stole  into  a  field  and  dug 
out  radishes.  A  postman  stopped  and  said,  "  Hello,  Bill," 
which  awakened  him  as  by  an  electric  shock.  A  barn  pre- 
sented itself,  in  which  he  spent  the  night.  In  the  morning, 
the  barn  looked  different.  In  fact,  his  entire  surroundings 
appeared  mysterious.  As  he  felt  like  working,  he  went  to 
work  in  front  of  the  barn. 

It  seems  that  in  his  life  there  had  been  two  other  episodes 
of  a  similar  nature;  in  fact,  Bachmann  had  been  in  a  state 
hospital  for  six  weeks  after  the  first  episode.  The  first 
episode  had  lasted  a  few  days  only,  and  followed  worry  when 
he  learned  that  the  girl  with  whom  he  was  in  love  was  married. 


PUZZLES   AND   ERRORS  265 

The  second  attack  followed  the  death  of  his  mother,  where- 
upon he  was  taken  to  a  state  hospital  although  the  total 
duration  of  symptoms  was  only  three  days.  Bachmann 
had  had  a  chancre  or  some  other  form  of  genital  disease  at 
26,  and  had  at  that  time  been  treated  with  mercury. 

Except  for  irregular  and  absolutely  rigid  pupils,  reacting 
neither  to  light  nor  to  accommodation,  Bachmann  showed 
no  physical  and  especially  no  neurological  disease  whatever. 
Moreover,  the  W.  R.  in  the  blood  serum  was  negative. 

As  to  diagnosis,  one  might  consider  hysteria,  of  which, 
however,  there  are  no  visible  stigmata.  It  would  not  appear 
that  brain  tumor  would  be  likely  to  have  lasted  so  long  as 
eight  or  nine  years,  even  if  we  should  attempt  to  make  the 
hypothesis  of  tumor  cover  both  the  non-reacting  pupils  and 
the  episodes.  Bachmann  was  non-alcoholic,  and  there  was 
no  sign  of  any  other  form  of  intoxication.  The  spinal  fluid 
showed  a  negative  gold  sol  reaction,  there  were  no  cells  in  the 
fluid,  there  was  no  globulin;  albumin  was  normal.  How- 
ever, the  W.  R.  was  strongly  positive. 

The  situation,  then,  in  this  case  is  that  we  have  somewhat 
peculiar  psychopathic  episodes,  pupils  rigid  to  light  and 
accommodation,  a  positve  W.  R.  in  the  spinal  fluid,  and  ex- 
tremely little  else  to  permit  a  diagnosis.  We  are  ignorant 
as  to  the  course  and  pathology  of  such  cases.  However,  we 
cannot  resist  the  temptation  of  the  diagnosis  of  neurosyphilis, 
although  further  classification  is  not  ventured. 

I.  What  is  the  significance  of  stiff  pupil  as  an  isolated 
symptom?  Nonne  finds  that  in  the  end,  after  years  of 
observation,  the  Argyll-Robertson  pupil  turns  put  to 
be  an  advance  courier  of  other  more  functionally 
serious  signs  and  symptoms  of  neurosyphilis.  We  can 
confirm  this  experience  and  regard  it  as  an  established 
clinical  proposition  that  the  Argyll- Robertson  pupil 
cannot  be  neglected.  In  this  connection,  refer  to  the 
case  of  alcoholic  pseudoparesis  (Murphy,  60),  and  also 
to  the  case  of  pineal  tumor  (Donald  Falvey,  35).  En- 
thusiastic reports  have  occasionally  been  made  upon 
apparent  restoration  of  the  true  syphilitic  Argyll- 
Robertson  pupil  to  normal  light  reaction.  ^  The  diffi- 
culties in  rendering  the  symptomatic  diagnosis  of 


266  PUZZLES  AND  ERRORS 

Argyll-Robertson  pupil  in  a  given  case  are  so  great,  and 
the  chances  of  complication  so  numerous,  that  we  are 
inclined  to  attach  little  significance  at  present  to  these 
claims. 

It  may  not  be  amiss  to  mention  a  somewhat  humorous 
incident  familiar  to  some  local  neurologists.  A  case  was 
reported  by  the  interne  for  a  number  of  months  as  a 
victim  of  a  pupil  stiff  to  light  and  accommodation,  and 
the  entirely  adequate  cause  of  this  phenomenon  was 
actually  only  discovered  at  autopsy  by  the  triumphant 
medical  examiner,  who  demonstrated  that  the  patient 
in  question  was  possessed  of  a  glass  eye. 


PUZZLES   AND   ERRORS  267 


TABETIC  NEUROSYPHILIS  ("  tabes  dorsalis  ") 
versus  PERNICIOUS  ANEMIA  with  spinal 
symptoms. 


Case  75.  Mrs.  Brown  was  a  woman  of  56,  who  for  the 
past  eight  or  ten  years  had  been  complaining  of  trouble  in 
her  legs.  As  she  described  it,  at  times  her  legs  were  so  weak 
she  could  hardly  stand;  at  other  times  there  was  consider- 
able pain  and  numbness.  She  has  always  been  considered 
"high  strung";  that  is,  she  had  a  very  bad  temper  and 
lost  control  of  herself  almost  entirely  when  she  became 
excited.  Her  legs  had  been  growing  progressively  worse, 
and  for  about  a  month  prior  to  admission  she  had  been  un- 
able to  stand  or  walk.  She  had  also  lost  control  of  her 
bladder.  On  account  of  her  temper,  it  had  been  almost 
impossible  to  nurse  her;  no  nurse  would  stay  with  her  be- 
cause of  her  scolding  and  fault-finding.  Recently,  she  had 
been  having  fits  of  the  blues. 

Her  husband,  who  was  seen  before  Mrs.  Brown,  was  an 
old  gentleman,  over  70,  who  was  chiefly  remarkable  from  the 
fact  that  he  had  unequal,  irregular  pupils,  which  reacted 
neither  to  light  nor  accommodation ;  there  was  also  a  speech 
defect. 

The  patient  herself  proved  to  be  extremely  irritable,  as 
had  been  stated,  —  so  much  so  that  at  times  it  seemed 
almost  impossible  to  do  anything  for  her.  She  was  very 
querulous,  constantly  complaining,  and  not  satisfied  with 
anything  that  was  done.  Aside  from  this,  her  mental  ex- 
amination proved  to  be  entirely  negative;  that  is,  there  were 
no  psychotic  symptoms. 

The  systematic  physical  examination  gave  the  following 
significant  findings:  blood  pressure,  160  systolic,  90  diastolic; 
no  evidences,  however,  of  peripheral  arteriosclerosis.  Patient 
was  unable  to  walk  or  stand,  and  had  no  control  over  her 
bladder.  The  knee-jerks  and  ankle-jerks  absent  on  both 
sides;  ataxia  in  the  leg  movements;  loss  of  sense  of  localiza- 


268  PUZZLES  AND  ERRORS 

tion,  with  no  tenderness  over  the  nerve  trunks;  no  atrophy, 
paralyses,  or  muscular  asymmetry  of  the  parts.  The  vibra- 
tory sense  was  maintained.  Subjectively,  the  patient  thought 
that  the  vibratory  sense  differed  in  the  legs  from  that  in  the 
arms.  Localization,  touch,  pain,  heat,  and  cold  responded 
to  correctly.  The  arms  showed  nothing  abnormal;  there 
was  no  incoordination,  dysmetria,  or  dysdiadochokinesis. 
Her  pupils  were  equal,  regular,  and  both  reacted  normally 
to  light  and  accommodation. 

Diagnosis :  The  first  consideration  in  the  case  is  naturally 
tabes  dorsalis,  especially  when  one  considers  that  the  hus- 
band had  signs  which  suggested  syphilis  of  the  nervous 
system.  The  rapid  onset  of  the  acute  symptoms  in  this 
case,  and  the  absence  of  the  characteristic  signs  of  pain  were 
at  least  atypical  for  this  diagnosis,  as  was  the  absence  of 
any  pupillary  signs.  Further,  the  W.  R.  was  negative  in 
the  blood  and  spinal  fluid;  there  were  no  definite  signs  of 
inflammatory  reaction  as  shown  by  the  other  spinal  fluid 
tests.  These  findings  made  a  diagnosis  of  tabes  entirely  im- 
probable. After  tabes,  the  most  frequent  cause  of  the  symp- 
toms above  enumerated  is  perhaps  to  be  found  in  pernicious 
anemia.  Examination  of  the  blood  showed  that  the  patient 
had  2,500,000  erythrocytes  per  cmm.  The  hemoglobin  by 
Tallquist  scale  was  80%.  The  smear  was  practically  nega- 
tive; no  blasts  could  be  seen.  Although  this  picture  is  not 
a  typical  one  for  pernicious  anemia,  at  least  it  is  significant 
in  the  low  number  of  red  cells  to  be  found,  and  as  no  causes 
for  anemia  were  to  be  found,  it  seemed  probable  that  we 
were  dealing  with  a  primary  anemia.  The  diagnosis  in  the 
case,  therefore,  is  spinal  sclerosis  of  primary  anemia.  The 
mental  picture  was  not  uncharacteristic  of  PERNICIOUS 
ANEMIA. 

I.  Could  the  diagnosis  be  rendered  in  this  case  without 
a  lumbar  puncture?  In  the  first  place,  the  emaciation 
is  not  entirely  characteristic.  The  pupils  react  nor- 
mally to  light.  Probably  such  a  case  might  well 
have  been  regarded  as  one  of  tabes  dorsalis  in  former 
days,  or  even  at  the  present  time,  if  a  lumbar  puncture 
had  not  been  resorted  to. 


PUZZLES  AND  ERRORS  269 

2.  Could  this  case  possibly  have  been  one  of  tabes  dorsalis 

with  negative  spinal  findings?  Such  cases  have  been 
reported  frequently,  but,  unlike  the  present  case,  are 
apt  to  be  of  long  standing  and  non-progressive,  in 
which  the  active  inflammation  is  no  longer  present. 
The  negative  findings  would  not  be  consistent  with 
tabes,  in  which  the  symptoms  are  of  short  duration 
and  of  sudden  onset. 

3.  If  the  serum  W.  R.  had  been  positive  would  the  diag- 

nosis have  been  neurosyphilis?  We  are  loath  to  make 
the  diagnosis  of  spinal  syphilis  when  the  spinal  fluid  is 
normal.  Syphilis  may  produce  a  marked  anemia, 
however,  and  thus  produce  symptoms  such  as  shown 
by  Mrs.  Brown.  It  is  even  possible  that  such  is  the 
explanation  of  this  case,  taking  into  consideration  the 
suggestive  findings  in  the  husband.  However,  there 
is  insufficient  evidence  to  make  such  an  hypothesis 
rock  firm,  and  we  do  not  more  than  suggest  it. 


270  PUZZLES   AND   ERRORS 


Atypical  case  of    CONGENITAL  NEUROSYPH- 
ILIS  —  peculiar  mental  state. 


Case  76.  James  Seabrook,  20  years  of  age,  obviously 
showed  a  number  of  signs  of  congenital  syphilis.  The 
physical  examination  disclosed  an  old  scar  and  indentation  in 
the  right  mastoid  region,  another  on  the  right  side  of  the 
neck,  another  on  the  posterior  surface  of  the  right  forearm, 
and  two  on  the  outer  surface  of  the  right  upper  arm.  The 
lesions  were  about  the  size  of  half  a  dollar.  There  was  a 
saddle-shaped  nose  and  a  perforation  of  the  palate  and 
uvula ;  there  were  palpable  cervical  and  axillary  glands,  small 
but  numerous.  There  was  a  dulness  in  the  region  of  the  right 
scapula,  and  slight  dulness  on  both  sides  behind.  There  were 
loud  whispering  and  piping  relies  and  bronchial  breathing 
throughout  the  chest,  more  marked  on  the  left;  there  was 
much  coughing,  and  the  sputum  was  at  times  blood-stained. 
The  pupils  were  irregular  but  reacted  perfectly.  The  left 
knee-jerk  was  slightly  more  active  than  the  right.  The  W. 
R.  in  blood  and  fluid  was  negative;  the  gold  sol,  globulin  and 
albumin  tests  were  negative.  There  were,  however,  56 
cells  per  cmm.  in  the  fluid. 

We  learned  that  the  patient  had  had  several  spells  of  great 
excitement,  with  pounding  on  the  door  and  a  desire  to  fight 
bystanders.  There  were  spells  of  headache  and  vertigo. 
Mentally  the  tests  showed  him  to  be  subnormal. 

The  diagnosis  of  CONGENITAL  SYPHILIS  seems  established ; 
possibly  the  pulmonary  condition  is  syphilitic.  The  mental 
subnormality  as  well  as  the  abnormal  traits  and  episodes 
are  probably  to  be  accounted  for  on  the  basis  of  syphilitic 
involvement  of  the  brain. 

1.  Are  the  headache  and  vertigo  connected  with  syphilis? 

This  is  perhaps  suggested  by  the  pleocytosis  in  the 
spinal  fluid. 

2.  How  shall  we  explain  the  negative  W.  R.?     This  patient 

had  received,  shortly  before  his  entrance  to  the  hospital, 


PUZZLES   AND   ERRORS  271 

salvarsan  and  mercury.  Possibly  the  drug  treatment 
has  little  or  nothing  to  do  with  the  negative  W.  R.'s 
since  they  not  infrequently  grow  weaker  as  congenital 
syphilitics  grow  older. 

What  is  the  explanation  of  the  spells  of  excitement? 
Compare  the  spells  of  excitement  in  a  form  of  neuro- 
syphilis  described  by  Kraepelin,  namely:  syphilitic 
paranoia,  discussed  in  the  case  of  Bridget  Collins  (59). 

Is  treatment  indicated  considering  the  W.  R.'s  to  be 
negative  in  blood  and  fluid?  Despite  the  negative 
jW.  R.'s  in  this  case  treatment  is  strongly  indicated 
on  account  of  the  pleocytosis.  This  would  seem  to 
indicate  that  there  is  an  active  inflammatory  process 
in  the  cerebrospinal  axis,  and  it  is  more  than  probable 
that  this  process  is  syphilitic.  How  much  real  im- 
provement of  the  symptoms  would  result  from  anti- 
syphilitic  treatment  it  is  impossible  to  prophesy. 
Every  case  is  a  special  problem,  and  this  case  is  very 
unusual  in  showing  a  pleocytosis  in  the  absence  of 
other  indications  of  syphilitic  nervous  disease,  viz., 
globulin,  albumin  and  W.  R.'s. 


272  PUZZLES  AND  ERRORS 


CONGENITAL  NEUROSYPHILIS  resembling 
an  undifferentiated  case  of  FEEBLEMINDED- 
NESS— actually  PARETIC. 


Case  77.  John  Friedreich,  a  y-year  old  boy,  was  brought 
to  the  Psychopathic  Hospital  by  agents  of  a  charitable  society, 
who  found  him  a  neglected  child  and  quite  evidently  a  sub- 
normal one. 

The  dominance  of  syphilis  in  the  situation  was  clear.  The 
boy's  father  had  died  but  a  few  months  before  of  syphilitic 
heart  disease,  from  which  he  is  said  to  have  suffered  for  five 
years.  The  boy's  mother  (the  parents  were  first  cousins) 
had  also  been  treated  for  syphilis  and  was  excessively  al- 
coholic. The  first  child  of  this  union  —  a  girl  —  had  died 
at  6  years,  of  a  disease  diagnosticated  spinal  meningitis. 
The  history  indicates  that  syphilis  was  acquired  after  the 
birth  of  this  first  child;  but  in  any  event  it  is  possible  that 
the  meningitic  condition  of  which  the  first  child  had  died  was 
syphilitic.  The  second  pregnancy  terminated  in  a  still- 
birth; the  third  issued  in  a  girl,  who  died  two  weeks  after 
birth  of  what  was  termed  "  inward  convulsions."  The 
fourth  pregnancy  resulted  in  a  miscarriage ;  the  fifth  in  our 
patient,  John  Friedreich.  The  sixth  pregnancy  resulted  in 
a  girl,  now  5  years  of  age,  who  is  apparently  normal.  (Her 
W.  R.  was  negative  and  she  shows  no  stigmata  of  syphilis.) 

The  patient,  John  Friedreich,  at  some  very  early  age  had 
a  rash  on  his  body  diagnosticated  as  syphilis.  He  also  had 
many  seizures  called  fainting  spells.  Ever  since  birth  he  had 
been  taking  mercury  pills.  He  had  not  learned  to  talk 
until  his  third  year,  and  was  able  then  to  say  only  a  few  dis- 
connected words.  In  fact,  John  has  never  been  able  to  talk 
in  complete  sentences,  mumbling  much  that  is  quite  unin- 
telligible. However,  he  walked  at  15  months  in  a  normal 
fashion  and  nothing  peculiar  in  his  gait  was  noted  until  he 
was  5  years  old,  when  he  began  walking  on  his  toes,  par- 
ticularly those  of  his  left  foot.  Shortly  thereafter,  the  seem- 


Juvenile  paresis.    7  years. 


PUZZLES  AND  ERRORS  273 

ingly  inevitable  trauma  appeared;  John  fell  out  of  a  window 
and  severely  injured  his  left  leg,  whereupon  the  peculiarity 
of  toe-walking  became  more  pronounced  and  associated 
with  a  limp. 

The  patient  strikes  one  physically  as  having  the  develop- 
ment of  a  child  of  about  five  years  (actual  age,  7).  There 
are  a  few  lymph  nodes  palpable  in  the  anterior  triangles  of  the 
neck.  The  dilated  and  slightly  unequal  pupils  react  neither 
to  light  nor  accommodation.  There  is  practically  complete 
deafness;  loud  sounds  are  not  at  all  noticed. 

Withal,  the  child  in  a  general  way  presents  a  somewhat 
attractive  appearance,  being  very  playful  and  mischievous, 
lying  about  on  the  floor  and  playing  with  whatever  comes  to 
hand,  talking  to  himself  or  making  a  few  indistinct  remarks 
to  the  bystanders.  He  walks  awkwardly,  on  the  toes  of  the 
left  foot.  He  pays  little  or  no  attention  to  his  toilet  and 
needs  to  be  dressed  and  cared  for  in  all  ways.  He  is  quick- 
tempered and  at  times  very  difficult  to  manage. 

There  was,  of  course,  little  doubt  of  the  diagnosis  of  CON- 
GENITAL SYPHILIS  and  of  FEEBLEMINDEDNESS.  The  W.  R. 
was  positive  both  in  the  blood  and  in  the  spinal  fluid.  The 
gold  sol  reaction  of  the  fluid  was  of  the  "  paretic  "  type; 
there  were  44  cells  per  cmm.  and  there  was  a  large  excess  of 
albumin  and  much  globulin. 

As  to  prognosis,  there  is  doubt. 

1.  Is,  or  is  mot,  this  a  case  of  juvenile  paresis? 

2.  Is   it,  perhaps,  a  relatively   permanent  case   of   feeble- 

mindedness due  to  congenital  syphilis?  On  the  whole, 
on  account  of  the  spinal  fluid  symptoms,  we  should  be 
inclined  to  give  the  case  a  relatively  poor  prognosis, 
namely,  of  death  in  a  few  years.  However,  we  may 
perchance  be  later  surprised  to  learn  that  the  patient 
has  lived  on,  at  least  into  early  adult  age. 

Note:  Mercury  tablets  in  some  cases  of  congenital 
syphilis  do  not  seem  effective.  John  Friedreich  was 
treated  most  intensively  by  syphilographers  from  birth. 

Dr.  W.  E.  Fernald  in  a  personal  communication  stated 
that  syphilitic  cases  of  feeblemindedness  are  rather  those 
of  the  imbecile  and  idiot  groups  than  of  the  higher  levels. 
This  statement  emphasizes  again  that  the  true  hereditary 


PUZZLES   AND    ERRORS 

cases  of  feeblemindedness  are  rather  those  of  the  higher 
group,  whereas  the  cases  in  which  special  causes  have 
operated  in  the  uterus  or  in  early  life  eventuate  in  idiocy 
and  imbecility.  However,  such  a  case  as  that  of  Fried- 
reich  shows  that  now  and  then  a  case  of  feebleminded- 
ness without  evidence  of  neurological  disorder  and  look- 
ing in  almost  all  respects  like  an  hereditary  case  may  be 
at  times  produced  by  syphilis. 

How  often  is  the  central  nervous  system  involved  in 
hereditary  syphilis?  An  interesting  table  bearing  on 
this  point  is  presented  by  Veeder.*  The  table  concerns 
the  lesions  in  various  parts  and  systems  of  the  body  in 
100  cases  of  late  syphilis.  It  appears  that  in  47,  or 
approximately  one-half  of  Veeder's  series  of  100  late 
cases,  the  infection  developed  some  form  of  lesion  of 
the  nervous  system.  As  Veeder  remarks,  this  result 
runs  counter  to  the  common  statements  of  pediatri- 
cians, notably  of  Holt. 


Bones: 

Periostitis  tibia 4 

Periostitis  skull I 

Osteomyelitis I 

Joints : 

Acute  arthritis  knee . .  8 

Acute  arthritis  ankle.  I 

Skin: 

Macular  eruption ....  I 

Condyloma  anus 3 

Gummata .'. .  3 

Alopecia 3 

Eye: 

Interstitial  keratitis.  .  24 

Choroiditis I 

Ulcerations: 

Nasal 2 

Laryngeal I 

Pharyngeal I 


Central  Nervous  System: 

Mental  deficiency 23 

Cerebrospinal  syphilis 14 

Hemiplegia 6 

Epilepsy 5 

Spastic  paraplegia 4 

Chorea 2 

Hydrocephalus 2 

Miscellaneous  Conditions: 

Ozena I 

Enlarged  spleen  (only  symp-  I 

torn) I 

Torticollis I 

Aortitis I 

Obscure  abdominal  pain ...  I 

Obscure  pain  in  legs 2 

Endarteritis  obliterans ....  I 

Paroxysmal  hemoglobinuria  I 

Raynaud's  disease I 

Hutchinson's  teeth 4 


*  Borden  S.  Veeder:  Hereditary  Syphilis  in  the  Light  of 
Recent  Clinical  Studies;  Am.  Jour,  of  Med.  Sc.,  CLII,  1916. 


PUZZLES   AND   ERRORS  275 


Juvenile  paretic  neurosyphilis.     Quadriplegia. 


Case  78.  Gridley  Ringer,  15  years  of  age,  had  the  facies  of 
a  congenital  syphilitic,  including  Hutchinsonian  teeth,  rhag- 
ades  of  the  face,  and  the  so-called  Olympic  brow.  No 
secondary  sexual  characteristics  had  developed.  There  was 
a  marked  speech  defect.  Mentally,  Ringer  was  a  low-grade 
imbecile.  He  had  been  born  at  full  term,  and  delivery  had 
been  normal.  There  had  never  been  other  pregnancies. 
He  had  never  developed  normally. 

The  father  admitted  syphilis  23  years  before,  namely, 
8  years  before  the  birth  of  his  son,  but  the  father  had  been 
treated  for  several  years  and  had  been  declared  cured. 

1.  What  would  be  expected  in  the  spinal  fluid  of  this  case? 

Without  the  history,  it  would  perhaps  be  impossible  to 
say  whether  the  case  was  one  of  a  quiescent  imbecility 
or  one  of  juvenile  paresis.  The  spinal  fluid  of  the  juve- 
nile paretic  gives  a  picture  identical  with  that  in  the 
adult.  The  spinal  fluid  in  this  case  showed  a  positive 
W.  R.  (as  did  also  the  serum),  a  marked  increase  of 
albumin  and  globulin,  115  cells  per  cmm.,  and  a  "  pa- 
retic "  gold  sol  reaction.  Accordingly,  the  diagnosis  of 
GENERAL  PARESIS  was  made. 

2.  What  is  the  prognosis?     The  prognosis  of  juvenile  paresis 

is  currently  regarded  as  entirely  grave.  There  is 
probably  less  hope  for  improvement  in  juvenile  paresis 
than  in  the  acquired  paresis  of  adult  life,  since  it  seems 
to  be  a  general  principle  that  congenital  syphilis  is 
always  more  difficult  to  cure  than  acquired  syphilis. 

This  case  had  seizures  a  few  months  after  initial 
observation,  and  the  seizures  were  followed  by  a  tran- 
sient right  hemiplegia.  This  right  hemiplegia  ^was 
shortly  followed  by  a  left  hemiplegia,  which  remained 
permanently.  Moreover,  a  few  weeks  later,  a  right 
hemiplegia  again  developed,  leaving  the  patient  with 
complete  paralysis  and  aphasia.  Death  followed  in 
six  weeks. 

3.  What  effects  were  shown  in  the  parents?     Following  up 

the  parents  was  rewarded  by  the  discovery  that  the 


276  PUZZLES  AND   ERRORS 

mother  was  suffering  from  nerve  deafness,  probably 
of  syphilitic  origin,  and  that  the  father  had  recently 
begun  to  suffer  from  what  he  considered  rheumatism, 
but  which  on  examination  was  shown  to  be  tabetic 
neurosyphilis  ("  tabes  dorsalis  ").  This  family  again 
supports  the  hypothesis  that  there  is  a  strain  of  spiro- 
chetes  especially  prone  to  attack  the  nervous  system. 
Here  it  would  seem  that  the  syphilis  acquired  by  the 
father  had  infected  the  mother  and  been  transmitted 
to  the  son.  In  all  three  infected  by  the  same  strain 
or  strains  of  organisms  the  nervous  system  was  in- 
volved. It  is  difficult,  nevertheless,  to  explain  on  this 
hypothesis  why  in  one  case  the  disease  took  the  form 
of  tabes  dorsalis,  in  the  second,  eighth  nerve  involve- 
ment and  in  the  third,  paresis.  This  question  of  strains 
is  really  more  than  academic  because  it  enters  deeply 
into  the  question  of  treatment,  as  well  as  that  of  the 
suggested  increased  viability  of  the  neural  strain. 


PUZZLES  AND  ERRORS  277 


Is  there  a  relation  between  epilepsy  and  juvenile 
neurosyphilis? 


Case  79.  John  Doran  fell  off  the  rear  of  an  ice-wagon,  at 
six  years  of  age,  and  shortly  afterward  developed  fits.  It 
appears  that  John  was  not  unconscious  at  the  time  of  his  fall, 
but  that  he  complained  of  headache.  Although  the  convul- 
sions were  fairly  frequent  at  first,  it  appears  that  they  later 
became  rare  and  occurred  only  when  the  patient  got  into  a 
temper.  At  the  stage  of  exhaustion  after  violent  excite- 
ment, John  would  fall. 

Physically,  at  9  years  a  fair  development  and  nutrition 
were  evident.  There  was  a  great  exaggeration  of  the  frontal 
bosses;  the  nose  could  not  be  said  to  be  typically  saddle- 
backed,  yet  there  was  a  suggestion  of  a  sinking  of  the 
bridge.  The  teeth  slightly  suggested  the  Hutchinsonian 
type,  but  only  slightly.  There  was  a  slight  roughening  of  the 
tibia,  and  there  was  a  slight  scar  over  either  knee.  The 
patient  graded  according  to  the  Binet  scale  at  9  years,  and 
he  was  regarded  as  definitely  feebleminded. 

The  family  physician  states  that,  according  to  his  informa- 
tion, the  father  contracted  syphilis  when  the  child  was  between 
three  and  four  months  of  age,  and  that  the  mother  also  was 
infected  at  this  time.  However,  the  child  had  not  been 
suckled  except  immediately  after  birth,  and  there  had  been 
no  evidences,  according  to  the  family  physician,  that  John 
had  acquired  syphilis. 

Ordinarily,  one  might  content  himself  regarding  the  case 
of  John  Doran  as  one  of  idiopathic  epilepsy  with  mental 
defect  or  deterioration.  However,  the  frontal  bosses,  sug- 
gestive teeth,  the  flattened  bridge  of  the  nose,  the  roughened 
tibiae,  and  the  old  scars,  though  singly  not  of  great  significance, 
collectively  make  one  suspicious.  Despite  the  family  physi- 
cian's belief  that  John  could  not  have  acquired  syphilis  from 
the  parents,  the  infection  seems  entirely  possible  despite  the 
fact  that  no  symptoms  developed  early  thereafter. 


378  PUZZLES   AND   ERRORS 

The  W.  R.  in  this  case  proved  positive  in  both  blood 
serum  and  spinal  fluid. 

1.  What  is  the  relation  of  trauma  to  this  case  of  JUVENILE 

NEUROSYPHILIS?     Probably  none. 

2.  What  would  be  the  effect  of  treatment?     For  a  number  of 

years  John  Doran  was  lost  sight  of.  He  was,  how- 
ever, treated,  according  to  our  information,  with  intra- 
spinous  injections  of  salvarsanized  serum,  whereupon 
his  convulsions  shortly  ceased.  He  has  been  recently 
examined  mentally  once  more,  and  still  grades  as  feeble- 
minded. He  still  has  violent  outbreaks  of  temper. 

3.  Is  such  a  case  as  Doran  typical  ?     Shanahan  has  investi- 

gated conditions  at  Craig  Colony.  There  were  22 
out  of  886  epileptics  (at  Craig  Colony)  or  2|%,  who 
showed  a  positive  W.  R.  Nine  of  these  cases  were 
regarded  by  Shanahan  as  cases  of  epilepsy  actually 
caused  by  syphilis.  Viet  had  found  7%,  and  Bratz  and 
Liith  5%  of  constitutional  epileptics  to  be  syphilitic, 
but  the  data  of  these  German  authors  were  obtained 
before  the  era  of  Wassermann  tests. 


PUZZLES   AND    ERRORS  279 


Adrenal  tuberculosis  complicating  juvenile  paretic 
neurosyphilis  ("juvenile  paresis").     Autopsy. 


Case  80.  When  James  Arnold  appeared  at  the  Danvers 
Hospital  in  his  22d  year,  he  looked  as  if  he  were  but  12  or  14 
years  of  age.  He  was  excessively  fat  but  of  fair  muscular 
development.  The  left  eye  diverged  outward,  and  the  left 
pupil  was  smaller  than  the  right.  An  odd  feature  was  a  high 
degree  of  pigmentation  of  the  skin  of  the  genitalia  and  the 
groins  (the  axilla,  the  mammillary  areas,  and  the  oral  mucosse 
were  free  from  pigmentation).  Physically  speaking,  the 
patient  was  practically  normal.  Neurologically,  however, 
there  was  much  of  interest,  in  the  light  of  which  the  clinical 
history  was  of  value. 

It  seems  that  after  an  apparently  normal  early  childhood, 
the  boy  had  begun,  at  the  age  of  n,  to  experience  difficulty 
in  carrying  out  every-day  school  tasks;  and  after  this  his 
mental  capacity  had  slowly  but  progressively  deteriorated. 
The  deterioration  was  not  merely  intellectual,  but  the  boy 
became  dishonest  and  untrustworthy  and  developed  a  number 
of  untidy  and  uncleanly  habits,  behaving  at  the  age  of  1 6,  as 
the  parents  stated,  like  a  child  of  six. 

In  his  seventeenth  year,  the  boy  had  been  taken  with  a  severe 
attack  of  what  was  regarded  as  an  "  attack  of  indigestion." 
This  attack  ushered  in  a  gradually  developing  muscular 
weakness,  especially  involving  the  limbs.  By  the  age  of  21 
he  had  become  irritable  and  the  paresis  was  so  extreme  that 
the  patient  was  unable  to  get  in  or  out  of  a  carriage. 

This  generalized  muscular  weakness  was  plain  upon  ad- 
mission to  the  hospital  though  there  seemed  to  be  no  actual 
paralysis.  The  patient  was  unable  to  walk  in  a  straight  line 
and  Romberg's  position  could  not  be  maintained.  Marked 
tremor  was  present  in  the  hands  and  lips.  There  was  bi- 
lateral impairment  of  vision  and  nystagmus.  Reflexes  and 
sensations  normal.  Speech  was  markedly  affected,  all  sylla- 
bles being  very  much  slurred.  School  knowledge  and  memory 


28O  PUZZLES   AND   ERRORS 

for  both  recent  and  remote  events  very  poor.  The  patient's 
habits  were  very  untidy.  He  was  very  emotional,  easily 
made  to  laugh  or  cry;  and  in  behavior,  extremely  childish. 

Two  months  after  his  admission  to  the  hospital,  the  weak- 
ness suddenly  became  extreme.  He  was  constantly  nau- 
seated, refusing  food.  The  face  and  hands  were  cyanosed  and 
the  heart's  action  rapid,  weak,  and  irregular.  This  attack 
lasted  for  a  week  and  was  followed  by  a  period  of  improve- 
ment, during  which,  however,  he  still  remained  very  weak  and 
apathetic. 

One  month  later  he  again  became  so  feeble  that  he  was 
unable  to  raise  himself  in  bed.  He  complained  persistently  of 
feeling  very  "  sick."  His  temperature  was  elevated  and  there 
occurred  the  same  train  of  circulatory  symptoms  observed 
previously,  viz.,  rapid  and  tumultuous  action  of  the  heart, 
with  cyanosis  of  face  and  extremities.  He  soon  became 
unconscious,  remaining  so  until  his  death,  which  occurred 
on  the  seventh  day  of  the  acute  attack. 

This  case  was  under  observation  before  the  days  of  the 
W.  R.,  yet  clinically  the  case  had  been  diagnosticated  JUVE- 
NILE PARESIS.  There  was  no  history  of  the  acquisition  of 
syphilis  or  any  likelihood  of  its  acquisition.  Considered  clini- 
cally, many  of  the  classical  features  described  by  Addison 
were  present,  viz.,  marked  asthenia  and  apathy;  severe  and 
frequent  gastro-intestinal  symptoms  (the  disease  probably 
commencing  with  the  attack  of  so-called  "  acute  indigestion  " 
six  years  prior  to  patient's  death) ;  attacks  of  extreme  cardiac 
weakness  with  the  characteristic  small,  low-pressure  pulse. 
On  the  other  hand,  pigmentation  of  the  skin  (usually  the 
most  striking  clinical  feature)  was  limited  to  the  external 
genitalia,  these  being  colored  a  deep  brown. 

The  most  striking  feature  found  at  autopsy  was  a  bilateral 
adrenal  tuberculosis  (caseation,  giant  cells,  lymphocytosis,  tu- 
bercle bacilli).  The  thymus  gland  was  persistent  (7X5X.5 
cm.),  whereas  the  thyroid  gland  was  smaller  than  usual. 
The  brain  showed  macroscopic  and  microscopic  features 
entirely  consistent  with  the  diagnosis  of  general  paresis, 
including  lymphocytosis,  plasmocytosis,  irregular  degrees  of 


PUZZLES   AND   ERRORS  28 1 

nerve-cell  destruction,  and  gliosis,  with  an  especially  charac- 
teristic microscopic  picture  in  the  frontal  regions. 

It  may  be  of  note  to  consider  the  degree  of  change  under- 
gone by  a  brain  in  1 1  years  or  more  of  deterioration,  and  the 
following  description  of  the  head  findings  is  therefore 
included : 

Head:  Hair  abundant,  dark.  Scalp  normal.  Cal- 
varium,  weight  435  gm.,  transparent  in  bregmatic  region 
only,  elsewhere  thick  and  dense.  The  average  thick- 
ness of  the  vertical  plate  of  the  frontal  bone  is  7  mm. 
The  frontal  bone  shows  a  moderate  thickening  and 
hardening  of  the  inner  table  with  obliteration  of  diploe. 
Dura  mater  moderately  adherent  to  the  bregmatic 
region  of  calvarium.  Arachnoidal  villi  moderately 
developed.  Sinuses  not  remarkable.  Pia  mater  shows 
a  moderate  focal  thickening  with  opacity,  especially 
along  sulci.  Vessels  well  injected.  Brain:  Weight, 
1 200  gm.  The  brain  shows  marked  focal  variations 
in  sulcation  and  consistence.  Spread  on  a  board,  the 
right  hemisphere  is  obviously  somewhat  bigger  than 
the  left.  There  is  a  difference  of  only  0.5  to  0.75  cm. 
on  measurement  of  the  greatest  circumference  of  the 
cerebrum,  taken  from  the  median  line  superiorly  to  the 
median  line  inferioriy,  but  the  right  hemisphere  is 
throughout  slightly  more  convex  than  the  left.  Both 
postcentral  gyri  are  much  narrowed  in  their  superior 
portions,  and  the  sulci  posterior  thereto  are  deeper 
than  the  other  sulci  of  the  hemispheres.  The  sulci 
of  the  orbital  surfaces  are  asymmetrical  and,  on  the 
left  side,  show  a  tendency  to  microgyria.  The  cerebral 
hemispheres  as  a  whole  show  a  remarkable  tendency  to 
slight  protrusion  of  the  border  gyri ;  especially  those  of 
the  two  poles,  of  the  free  edges  along  the  great  fissure, 
and  most  strikingly  the  gyri  at  the  boundary  line  between 
the  inferior  and  lateral  surfaces.  This  marginal  prom- 
inence is  slight  but  obvious  and  is  emphasized  by  a 
slightly  paler  color  in  some  regions.  The  cerebrum 
shows  a  general  induration  which  is  greatest  in  the  frontal 
tips  and  along  the  inferior  borders  of  the  lateral  surfaces 
of  the  hemispheres,  especially  right.  The  orbital  sur- 
faces are  firm,  especially  anteriorly  and  externally  (pre- 
frontal);  the  tips  of  the  temporal  lobes  are  firm,  and 
the  superior  temporal  gyri  are  firmer  than  adjacent 
gyri.  The  postcentral  gyri  are  indurated  more  than 


282  PUZZLES   AND    ERRORS 

the  other  gyri  of  the  superior  surface.  The  hippo- 
campal  gyri  are  likewise  firmer  than  adjacent  gyri. 

Cerebellum  and  pons:  Weight,  145  gm.  The  in- 
equality of  the  two  hemispheres  is  more  marked  than  in 
the  case  of  the  cerebrum. 

Greatest  lateral  diameter;   left,  4.5  cm.,  right,  5.5  cm. 

Anteroposterior  diameter  adjacent  to  notch:  Left, 
5.8  cm.,  right,  5.5  cm. 

There  is  no  appreciable  difference  in  depth.  The 
diminution  in  volume  appears  to  be  chiefly  at  the  expense 
of  the  right  clivus.  The  inferior  surface  is  firmer  than 
the  superior.  The  laminae  adjacent  to  the  horizontal 
fissure  are  firmer  than  the  remainder  of  the  cerebellum. 
The  pons  is  small. 

There  was  also  a  lateral  curvature  of  the  spinal  column, 
as  well  as  characteristic  adhesions  between  spinal  dura  and 
pia  mater  which  are  always  suggestive  of  syphilis.  For  the 
rest,  there  were  few  findings  of  note:  some  adhesions  of  the 
left  pleura,  hypostatic  congestion  of  the  lungs,  tracheitis,  and 
chronic  gastritis.  There  were  four  lobes  of  the  right  lung 
but  it  is  doubtful  whether  this  should  be  regarded  as  a  stigma. 

1.  Can  we  separate  the  symptoms  of  Addison's  disease  from 

those  of  paresis  in  this  case?  The  extreme  cardiac 
weakness  with  a  characteristic,  small  low-pressure 
pulse  is  in  point.  The  asthenia  and  apathy  are  consis- 
tent enough  with  Addison's  disease  as  well  as  with  paresis 
itself.  It  would  also  be  possible  to  ascribe  the  gastro- 
intestinal symptoms  to  either  disease. 

2.  Of  what  significance  is  the  persistent  thymus?     Persistent 

thymus  has  been  observed  in  a  few  cases  of  Addison's 
disease,  but  that  it  plays  any  part  in  the  symptoma- 
tology thereof  is  a  matter  of  doubt. 

3.  How  can  the  obesity  be  explained?     It  is  of  course  of 

note  that  the  thyroid  gland  was  small,  but  micro- 
scopically there  were  no  peculiar  features  in  this  gland. 

4.  Was  the  adrenal  tuberculosis  actually  primary?     Minute 

search  failed  to  reveal  evidences  of  tuberculosis  else- 
where unless  we  regard  the  few  adhesions  binding  the 
lower  half  of  the  lung  to  the  chest  wall  as  indicative 
of  an  old  tuberculosis.  In  particular,  the  mesenteric 
lymph  nodes  were  normal. 


PUZZLES   AND   ERRORS  283 


Neurosyphilis?    Secondary  stage  of  syphilis. 


Case  81.  Florence  Fitzgerald,  a  woman  25  years  of  age, 
applied  at  the  police  station  to  be  taken  care  of.  She  said 
she  had  been  a  prostitute  for  the  last  few  months,  was  now  ill, 
and  wanted  to  reform.  She  appeared  physically  ill  and  was 
sent  to  the  Psychopathic  Hospital,  where  she  remained  at 
first  almost  mute,  making  answers  chiefly  by  nodding  the 
head.  She  gave  the  impression  of  daze  or  stupor,  and  in 
fact  her  condition  was  at  first  regarded  as  catatonic.  This 
reaction,  after  a  few  days,  changed  and  Florence  became 
quite  normal,  giving  a  full  account  of  her  condition. 

It  seems  that  four  months  before  going  to  the  police  station, 
she  developed  a  chancre,  which  was  locally  treated.  A 
careful  physical  examination  showed  a  fine  red  macular 
eruption  which  was  without  much  question  a  syphilitic 
roseola.  The  spinal  fluid  yielded  a  positive  W.  R.  although 
other  tests  of  the  fluid  were  negative.  Curiously  enough,  no 
physical  sign  of  involvement  of  the  nervous  system  could  be 
discovered.  We  were  inclined  to  regard  the  mental  symptoms 
as  partly  due  to  the  syphilitic  intoxication,  and  partly  due  to 
a  psychic  reaction  of  the  nature  of  defense.  As  for  the  posi- 
tive W.  R.  in  the  spinal  fluid,  in  early  secondaries  various 
observers  differ  as  to  the  frequency  both  of  the  W.  R.  and 
of  other  changes,  percentages  being  given  that  range  from 
25  to  90%.  See  case  Caperson  (45).  It  is  of  note  that 
clinically  there  were  symptoms  referable  to  a  syphilitic  in- 
volvement of  the  nervous  system ;  namely,  marked  headache 
and  malaise.  The  headaches  of  the  secondary  period  are  fre- 
quently the  result  of  meningeal  involvement. 


284  PUZZLES   AND   ERRORS 


TABOPARETIC  NEUROSYPHILIS  ("  tabo- 
paresis  ") ;  death  from  TYPHOID  MENINGITIS. 
Autopsy. 


Case  82.  Frederick  Estabrook  was  a  salesman,  who,  be  it 
noted,  had  never  had  typhoid  fever  or  any  disease  remotely 
resembling  typhoid  fever.  He  had  acquired  syphilis  at  19; 
had  married  at  22;  was  the  father  of  two  healthy  children 
(no  miscarriages) ;  had  had  a  certain  disturbance  of  bladder 
and  rectum,  but  remained  a  successful  salesman  to  the  age  of 
28,  when  advancing  tabes  confined  him  to  bed  for  a  time. 
At  30,  mental  signs  of  PARETIC  NEUROSYPHILIS  developed, 
and  death  followed  at  32,  after  an  acute  illness  of  a  week. 

The  details  of  the  history  after  the  first  symptoms  at  28 
are  as  follows: 

At  twenty-eight  patient  lost  control  of  limbs  and  was  con- 
fined to  the  house  about  two  months,  under  medical  care. 
Three  months  later  he  had  regained  partial  control  of  his 
limbs  but  had  lost  all  control  of  his  sphincters.  After  another 
month  he  had  returned  to  work,  but  did  not  work  steadily 
and  seemed  to  have  lost  ambition.  In  the  summer  of  1905, 
his  mind  became  obviously  altered.  He  grew  indolent  and 
extravagant  and  given  to  buying  expensive  and  useless  ar- 
ticles. Loss  of  interest  in  things  followed,  together  with  loss 
of  memory  for  recent  events,  lack  of  insight  into  illness,  delu- 
sions of  persecution  by  wife,  irascibility  followed  quickly  by 
crying.  Before  admission  to  hospital,  he  was  euphoric,  drawl- 
ing and  tremulous  in  speech,  sprawling  in  penmanship,  alter- 
nately depressed  and  exalted  in  manner.  Knee-jerks  were 
absent,  gait  ataxic,  pupils  stiff  to  light. 

The  family  history  was  negative  with  respect  to  insanity. 
All  the  family  were  reported  as  nervous.  A  brother  died  of 
peritonitis  at  twenty-eight,  a  sister  of  pneumonia  under  twenty. 
Another  brother  and  sister  are  living.  Father  and  mother  died 
of  heart  trouble  at  about  sixty-seven  and  sixty  respectively. 

The  patient  was  at  high  school  one  year  and  was  a  fair 


PUZZLES  AND  ERRORS  285 

student.  Considerable  tobacco  was  used,  and  some  alcohol. 
Intoxication  denied.  There  was  no  history  of  typhoid  fever 
or  other  acute  disease. 

The  patient  on  admission  was  sallow,  poorly  nourished,  and 
flat-chested,  with  a  slight  lateral  curvature.  There  was 
slight  dulness  over  right  apex  in  front  and  in  right  upper 
back.  Voice  sounds  were  increased  over  right  apex  in  front 
and  over  whole  right  back.  The  right  chest  showed  bronchial 
respiration  throughout.  The  respiration  in  front  of  right  chest 
was  of  an  interrupted  character.  The  liver  seemed  moder- 
ately enlarged.  The  urine  showed  a  very  faint  trace  of 
albumin.  There  were  a  few  small  nodes  in  right  groin  and  a 
scar  on  dorsum  of  penis. 

Neurological  Examination.  Slight  swaying  in  Romberg 
position.  Slight  tremor  of  protruded  tongue  and  extended 
fingers.  Pupils  irregular,  left  slightly  larger  than  right.  Left 
pupil  reacted  to  light  consensually,  but  not  directly.  Right 
pupil  reacted  very  slightly  to  direct  light,  not  consensually. 
Knee-jerks  and  Achilles  jerks  absent.  Ankle  clonus  absent, 
abdominal  and  cremasteric  reflexes  brisk.  Sharp  and  dull 
points  were  recognized  in  the  legs  with  numerous  mistakes. 
Vocal  and  facial  tremor.  Speech  slow  and  drawling.  Test 
phrases  repeated  well  if  care  was  taken.  Consciousness  clear. 
Orientation  perfect.  Calculating  ability  preserved.  Many 
words  omitted  in  writing.  Penmanship  clear  but  shaky. 

Hallucinations  absent.  Memory  of  recent  events  poor. 
Associations  of  a  logical  or  defining  type.  Patient  denied 
various  statements  in  commitment  papers  and  had  little  or  no 
insight  into  the  mental  side  of  his  disease  —  slight  euphoria. 

After  a  month's  observation  the  patient  was  removed  to  a 
quiet  ward  and  set  to  work  a  few  days  in  the  scullery.  One 
night  he  began  to  yell  as  if  assaulted  and  said  later  that  he 
had  an  idea  that  he  was  going  to  die.  Before  three  months 
had  passed  he  had  become  untidy,  disorderly,  and  imperfectly 
oriented. 

The  general  degeneration  continued  rapidly.  One  week 
before  death  the  temperature  rose  to  103  degrees  F.,  and  the 
patient  succumbed  to  what  seemed  clinically  like  a  broncho- 
pneumonia.  Unconsciousness  two  days  before  death. 


286  PUZZLES   AND   ERRORS 

Note  with  respect  to  history  of  typhoid.  —  Inquiries  of 
his  physicians,  wife,  employer,  and  brother  tend  to  show 
conclusively  that  the  patient  never  had  a  disease  even  re- 
motely resembling  typhoid  fever. 

The  autopsy  findings  were  as  follows: 

Acute  conditions: 

Hypostatic  pneumonia,  with  early  serofibrinous  pleuritis 
and  without  lymph  node  swelling ;  enlargement  of  mesenteric 
lymph  nodes;  acute  cerebrospinal  leptomeningitis;  multiple 
small  hemorrhages  of  spleen. 

Other  findings: 

Scar  of  penis;  sclerosis  of  aortic  arch  (Heller's  type?)  and 
slight  coronary  arteriosclerosis;  calvarium  thin  and  dense; 
dura  mater  thickened  and  adherent  to  calvarium;  calcified 
arachnoidal  villi;  chronic  cerebral  and  cerebellar  leptomen- 
ingitis; atrophy  of  frontal  lobes ;  granular  ependymitis;  scle- 
rosis of  posterior  columns  of  spinal  cord ;  emaciation ;  unequal 
pupils;  slight  parietal  fibrous  endocarditis,  slight  mitral  scle- 
rosis; gastrointestinal  atrophy;  chronic  cystitis;  chronic  ab- 
scess of  prostate. 

The  description  of  the  head  findings  is  as  follows : 
Skin  exceedingly  loose,  and  the  whole  skull  cap 
thinned.  The  diploe  are  absent.  Adhesion  with  dura 
easily  separated.  The  dura  somewhat  thickened,  but 
not  distended.  Along  the  longitudinal  sinus  extensive 
calcareous  granulations  adhere  to  it.  The  longitudinal 
sinus  does  not  contain  blood,  and  the  inner  surface 
is  normal  in  color.  The  pia  is  extensively  thickened 
and  opaque  and  a  general  subpial  exudate  exists  which 
is  more  marked  over  the  vertex  where  it  lifts  the  pia 
from  the  brain  surface  to  the  extent  of  three  centi- 
meters in  Rolandic,  superior  frontal,  intraparietal, 
and  mesial  precentral  sulci  on  each  side.  The  arteries 
at  base  are  free  from  atheroma.  The  temporal  lobes 
are  much  bound  down  by  adhesions,  as  is  the  cerebellum. 
Post-mortem  softening  is  evident.  The  hemispheres 
show  no  asymmetry,  but  the  frontal  convolutions  are 
markedly  atrophic.  The  corpus  callosum  is  united  to 
the  cortex  by  old  adhesions  and  has  to  be  dissected 
away  from  it.  Lateral  ventricles  contain  some  slight 
amount  of  cloudy  fluid,  and  the  pia  along  the  vessels 


PUZZLES   AND   ERRORS  287 

is  opaque.  Some  granulations  in  ependyma.  Brain 
weight,  1305  grams.  Pons  and  cerebellum,  195  grams. 

Cord.  —  Dura  much  thickened,  and  the  pia  corre- 
sponds to  its  appearance  in  brain  with  a  like  exudate. 
Cross-sections  of  cord  show  sclerosis  of  posterior 
columns. 

Bacteriologically  the  typhoid  bacillus  was  cultivated 
from  the  meninges  and  from  the  swollen  mesenteric  lymph 
nodes.  The  blood  was  negative;  the  intestines  were 
negative  so  far  as  lesions  were  concerned. 

The  microscopic  examination  confirmed  the  clinical  diag- 
nosis of  GENERAL  PARESIS  and  of  TABES,  since  there  was 
not  only  an  extensive  chronic  encephalitis,  with  the  usual 
lymphocytic  and  plasma-cell  deposit  and  irregular  gliosis, 
but  also  a  well-marked  posterior  column  sclerosis,  not  un- 
usual save  in  its  extreme  degree. 

It  might  be  surmised  that  some  difficulty  would  arise  in 
distinguishing  the  effects  of  paretic  meningoencephalitis  from 
those  of  the  more  recent  typhoidal  process.  The  well-known 
tendency  of  typhoidal  processes  to  escape  polynuclear  ex- 
udation, at  least  until  frank  necrosis  has  set  in,  gave  rise  to 
the  idea  that  the  two  mononuclear  pictures  —  that  of  general 
paresis  and  that  of  typhoidal  processes  —  might  be  confusing. 

The  picture  presented  by  the  meninges  was  scarcely  what 
might  be  expected.  Although  numerous  mononuclear  phag- 
ocytic  cells  are  everywhere  found,  yet  the  predominant 
picture  is  that  of  a  polynuclear  exudation. 

The  polynuclear  leucocytes  occur  in  greatest  numbers  in 
the  tissue  spaces,  especially  in  the  meshes  of  the  lumbar  arach- 
noid and  in  the  spaces  of  the  frontal  and  paracentral  pia 
mater.  In  the  lumbar  region  of  the  spinal  arachnoid  wide 
fields  occur  in  which  the  cells  are  almost  one  hundred  per 
cent  polynuclear  leucocytes.  In  places  phagocytic  cells  oc- 
cur, and  in  a  few  fields,  even  in  the  open  tissue  spaces,  the 
number  of  phagocytic  cells  may  arise  to  fifty  per  cent. 
Edema  is  a  considerable  feature  in  the  meninges.  Fibrin  is 
found  chiefly  in  the  cerebral  meninges  and  appears  in  numer- 
ous delicate  strands  in  the  tissue  spaces. 


Moloch,  horrid  king,  besmeared  with  blood 

Of  human  sacrifice,  and  parents'  tears; 

Though,  for  the  noise  of  drums  and  timbrels  loud, 

Their  children's  cries  unheard  that  passed  through'fire 

To  his  grim  idol. 

Paradise  Lost,  Book  I,  lines  392-396 


IV.   MEDICOLEGAL  AND   SOCIAL. 


Neurosyphilis  in  a  public  character:    eloquence, 
reformatory  efforts,  notoriety. 


Case  83.  Major  Isaac  Thompson,  M.D.,  was  a  character. 
He  had  been  regarded  as  eccentric  for  many  years  prior  to 
his  death  at  63.  In  fact,  it  seems  that  there  had  been  more 
or  less  definite  symptoms  and  signs  about  his  fortieth  year. 
The  doctor  himself  had  a  ready  explanation  for  his  Argyll- 
Robertson  pupils;  he  explained  that  he  had  had  a  peculiarly 
heavy  smallpox  at  about  the  age  of  27  (which  would  be 
about  1872). 

The  doctor  had  a  good  secondary  education,  he  had  gone 
through  the  Civil  War  as  a  hospital  steward,  went  into  busi- 
ness after  the  war,  married,  and  then  went  to  the  medical 
school,  graduating  at  the  age  of  34.  He  continued  in  prac- 
tice for  a  dozen  years,  and  then  gave  it  up.  For  years  he 
had  been  especially  interested  in  certain  literary  lines  and 
he  had  published  any  number  of  pamphlets,  all  of  a  some- 
what striking  description,  often  with  a  political  color  and 
intended  to  stir  up  reform  measures.  The  doctor  never  bore 
a  very  good  reputation,  and  years  later  it  was  recalled  that 
certain  books  disappeared  from  libraries  and  their  loss  was 
almost  certainly  traced  to  Dr.  Thompson.  In  general,  how- 
ever, he  was  considered  to  be  a  rather  worthy  local  figure. 

It  is  possible  that  a  fall  on  the  ice  in  his  6ist  year  actually 
started  the  fatal  process,  since  after  that  time  the  patient 
had  difficulty  in  walking,  and  a  few  months  later  developed 
periods  of  excitement  with  peremptory  insistence  on  obedi- 
ence to  his  wishes.  Whereas  formerly  the  doctor  had 
finished  up  one  literary  piece  of  work  after  another,  he  now 
began  to  do  very  scattering  work.  He  appeared  in  public 

289 


MEDICOLEGAL   AND   SOCIAL 

to  denounce  certain  financial  schemes  with  great  force  and 
unusual  eloquence.  His  eloquence  was  greatly  compli- 
mented, and  these  compliments  induced  the  doctor  to  a 
remarkable  crusade  against  a  certain  corporation;  there  was 
so  much  truth  mixed  with  the  fiction  of  his  eloquence  that 
he  obtained  a  considerable  following  in  his  campaign.  He 
wanted  to  start  a  bureau  of  information  for  the  instruction 
of  the  public  on  these  matters,  and  he  planned  to  put  up  a 
building  adjoining  his  own  home  for  the  accommodation  of 
the  various  clerks  and  writers  in  this  bureau.  However, 
before  the  building  had  been  actually  started,  an  outbreak 
occurred. 

One  morning  the  doctor  was  very  excitable  and  noisy  over 
the  telephone,  ordering  typewriters  and  giving  directions  to 
mechanics.  He  repaired  to  Boston  in  connection  with  cer- 
tain resources  that  he  supposed  (and  gave  others  reason  to 
believe)  had  been  supplied  by  the  Government  and  by  a 
large  newspaper.  One  evening  he  returned  very  late.  It 
appeared  that  he  had  had  a  fracas  at  a  hotel  and  had  knocked 
down  one  or  two  colored  porters,  acting  as  though  drunk. 
Upon  being  put  to  bed,  the  doctor  talked  incessantly  of 
religious  matters,  proposing  to  undertake  a  Sunday  School 
class.  His  interlocutor  did  not  exhibit  a  particular  interest 
in  this  scheme,  whereupon  Dr.  Thompson  threatened  him 
with  violence.  Police  and  doctors  were  called  in  and  a  con- 
stant stream  of  conversation  lasted  for  hours.  The  patient 
was  finally  brought  to  Danvers  Hospital  upon  representa- 
tion by  physicians,  to  whom  he  told  that  his  luck  had 
turned,  that  he  was  about  to  be  made  senator  from  the 
district,  and  that  he  and  Roosevelt  were  going  to  break  up 
the  trusts,  and  that,  as  a  matter  of  fact,  he  was  a  relative 
of  Mr.  Roosevelt. 

Upon  admission,  the  patient  was  a  well  preserved  and 
well  groomed  man  with  gray  hair  and  beard.  He  was  some- 
what pallid  but  his  teeth  were  well  preserved  and  well  cared 
for,  and  there  was  little  or  no  physical  change  except  a  slight 
hypertension.  He  claimed  that  he  had  suffered  from  kidney 
disease  for  some  years,  and  there  was  in  fact  a  trace  of 
albumin  in  the  urine. 


MEDICOLEGAL  AND   SOCIAL  29! 

Neurologically,  the  plantar  and  Achilles  reactions  could 
not  be  obtained,  but  there  were  no  other  reflex  disorders 
except  the  bilateral  Argyll-Robertson  pupil.  The  doctor's 
explanation  for  these  stiff  pupils,  which  he  described  as 
existing  for  many  years,  was  frank  and  circumstantial,  so 
that  the  unlikelihood  of  Argyll-Robertson  pupils  due  to  small- 
pox was  rather  frowned  upon  by  him.  Without  entering  upon 
a  detailed  description  of  the  clinical  symptoms  and  course 
of  the  disease  which  led  to  death  a  little  over  a  year  after 
admission,  it  may  be  said  that  the  differential  diagnosis  lay 
between  the  expansive  form  of  general  paresis  and  a  maniacal 
condition,  presumably  the  maniacal  phase  of  manic-depres- 
sive psychosis.  From  the  data  of  a  special  staff  meeting 
held  upon  the  case,  we  learn  that  the  diagnosis  of  manic- 
depressive  psychosis  was  entertained  more  strongly  than  that 
of  general  paresis.  Thus,  for  general  paresis  alone  was  the 
somewhat  gradual  onset  with  increasing  excitement,  accom- 
panied by  expansive  delusions  concerning  unlimited  finance, 
personal  over-importance,  and  Argyll-Robertson  pupils. 
Dismissing  the  Argyll-Robertson  pupils  from  consideration, 
the  diagnosticians  were  led  to  see  in  the  constant  motor 
activity  displayed  in  conveying  an  enormous  number  of 
thoughts  on  paper,  inconsistent  talking  with  digressions,  a 
manic-depressive  psychosis.  There  was  no  amnesia  and  no 
other  sign  of  mental  deterioration.  There  was  a  certain 
improvement  early  in  the  hospital  stay  of  the  patient. 
Consciousness  was  clear  and  orientation  perfect.  The  de- 
lusions themselves,  though  extravagant,  were  not  inconsis- 
tent or  fantastic.  The  hallucinatory  disorder  was  hardly 
characteristic  either  of  manic-depressive  psychosis  or  of 
paresis. 

The  patient  might  be  described  as  "  interesting."  A 
good  preliminary  training  with  years  of  travel  and  variety 
of  occupation,  furnished  him  with  a  fund  of  knowledge. 
An  excellent  memory,  prompt  replies  and  repartee,  endless 
digressions  with  voluntary  return  to  the  original  topic, 
caused  him  to  be  an  amusing  and  even  instructive  inter- 
locutor. However,  his  commitment  and  confinement  in  the 
institution  seemed  always  entirely  wrong,  and  he  expressed 


292  MEDICOLEGAL  AND   SOCIAL 

mixed  feelings  about  the  family,  now  being  bitter  against 
them,  and  again  condoning  their  mistakes.  The  patient's 
conduct  was  good  and  he  was  tidy  in  habits,  and  tried  as 
far  as  possible  to  conform  to  the  requirements  of  the  hos- 
pital. The  doctor  showed  a  marked  antipathy  toward  a 
certain  male  attendant,  who  had  removed  articles  from  his 
clothing  upon  admission  and  had  reclaimed  a  book  on  rules 
and  regulations.  The  doctor  prepared  a  list  of  327  differ- 
ent acts  of  abuse,  lack  of  care,  and  insubordination  which 
he  said  he  had  observed  in  the  hospital. 

In  the  last  weeks  of  the  patient's  illness,  his  ideas  became 
more  expansive  and  extravagant,  dealing  with  a  grapevine 
system  of  wireless  communication  and  delusions  of  un- 
limited wealth.  He  would  at  times  keep  his  room  flooded 
with  urine  and  water  for  the  purpose  of  keeping  down  the 
plague  which  he  said  was  infecting  the  hospital.  Later  he 
mixed  food  with  urine  and  other  ingredients,  claiming  that 
he  was  constructing  an  elixir  of  life. 

The  autopsy  showed  few  changes  of  the  calvarium  or  of 
the  dura  mater,  nor  was  the  pia  mater  more  than  slightly 
thickened  and  milky  over  the  frontal  poles,  along  the  longi- 
tudinal fissure  and  over  the  sulci.  There  were  fairly  firm 
adhesions  of  the  pia  mater  to  the  dura  mater  along  the 
longitudinal  fissure  and  over  the  frontal  poles  and  at  the 
temporal  tips.  The  hemispheres  were  firmly  interadherent, 
and  the  cerebello-pontine  tissues  were  covered  with  a  firm 
leptomeningitis.  The  floors  of  the  ventricles  were  smooth 
and  the  basal  vessels  showed  little  beyond  a  few  spots  of 
sclerosis.  There  was  a  generalized  increase  of  consistence. 
The  frontal  gyri  were  rather  prominent  with  wide  sulci,  but 
upon  section  no  very  marked  atrophy  of  the  gray  matter 
could  be  shown.  The  rest  of  the  brain  failed  to  show  any 
flaring  of  sulci  or  any  special  evidence  of  cortical  atrophy. 
The  brain  weighed  1250  grams;  a  possible  diminution  of  100 
grams,  considering  the  patient's  body  length.  However,  it 
must  be  remembered  that  he  was  at  this  time  63  years 
of  age. 

Microscopically,  the  diagnosis  of  GENERAL  PARESIS  was 
confirmed  on  the  basis  of  plasmocytosis,  lymphocytosis,  gliotic 


MEDICOLEGAL  AND   SOCIAL  293 

changes  and  nerve-cell  destruction.  There  was  an  unusual 
variation  in  the  degree  of  the  destructive  process,  which 
picked  out,  for  example,  certain  regions  of  the  right  side 
for  maximal  lesion  (cornu  ammonis,  gyrus  rectus,  and  su- 
perior frontal  gyrus). 

If  the  patient's  own  estimate  of  35  years'  duration  for 
his  Argyll- Robertson  pupils  can  be  trusted  (and  in  general 
his  memory  was  extremely  good),  we  may  well  conceive  an 
unusual  duration  for  the  process  in  his  case.  There  was, 
however,  in  the  body  at  large  no  very  marked  degree  of 
changes.  There  was  a  slight  old  tuberculosis.  There  was 
a  slight  interstitial  nephritis,  with  cardiac  hypertrophy  and 
fibrous  myocarditis.  There  was  also  a  sclerosis  of  the  mitral 
and  aortic  valves;  there  were  chronic  changes  in  the  spleen, 
liver,  and  bladder;  there  was  generalized  arteriosclerosis  of 
mild  degree;  there  were  two  round  gastric  ulcers  near  the 
pylorus.  The  liver  weighed  but  800  grams,  and  its  left 
lobe  was  somewhat  rough. 

This  case  is  placed  among  the  medicolegal  and  social 
cases  because  the  phenomena  that  ushered  in  his  last  illness 
were  mistaken  by  the  local  public  for  meritorious  social 
reform  measures.  They  were  regarded  as  not  markedly 
different  from  the  variety  of  steps  taken  by  the  very  active 
doctor  in  previous  years;  indeed  the  public  eloquence  that 
he  displayed  a  year  before  his  death  was  quite  in  line  with 
previous  habits,  despite  the  suspicious  over-brilliance  of 
language.  It  is  an  important  question,  how  far  the  eccen- 
tricity and  literary  overactivity  of  the  latter  half  of  the 
doctor's  total  life  can  be  explained  on  the  basis  of  a  mild 
syphilitic  irritation  of  the  nervous  system.  In  this  con- 
nection we  are  tempted  to  recall  the  suggestions  of  Mcebius 
concerning  a  portion  of  the  literary  products  of  Nietzsche. 
Our  doctor  was  by  no  means  so  brilliant  an  exemplar  of 
syphilitic  literature  as  was  Nietzsche,  if  we  grant  the  hy- 
pothesis of  Mcebius  to  cover  our  doctor's  case  as  well  as 
that  of  Nietzsche.  In  the  future,  important  studies  of 
character  change  under  the  influence  of  syphilis  will  doubt- 
less be  made.  With  modern  diagnostic  methods,  of  course, 
the  diagnosis  would  have  been  rendered  almost  at  once  in 


294  MEDICOLEGAL   AND   SOCIAL 

the  case  of  Major  Isaac  Thompson,  M.D.,  and  much  of  his 
past  life  would  have  been  brought  under  special  review  in 
connection  with  the  syphilis  which  doubtless  the  blood 
serum  or  at  any  rate  the  cerebrospinal  fluid  would  have 
shown. 

This  case  illustrates  but  one  of  the  many  social  compli- 
cations arising  as  the  result  of  paresis.  When  one  recalls 
that  the  onset  is  often  insidious  and  not  correctly  under- 
stood for  a  period  of  time,  it  is  readily  seen  that  many  unfor- 
tunate acts  may  be  committed  by  a  patient.  As  hypersexual 
desire  is  not  an  infrequent  early  symptom  and  as  judgment  is 
early  disturbed,  loose  morals  may  ruin  the  patient's  reputation. 
The  poor  judgment  and  expansive  delusions  often  lead  to 
foolish  business  deals  wherein  the  patient's  family  is  left 
destitute.  At  other  times  the  onset  is  sudden  and  then  the 
danger  of  false  commands  or  acts  by  a  person  in  a  respon- 
sible position,  as  a  steamship  captain,  an  engineer  or  chauffeur, 
may  lead  to  loss  of  life  and  property. 


MEDICOLEGAL   AND   SOCIAL  295 


Sudden   grandiosity:    debts.      PARETIC    NEURO- 
SYPHILIS  ("general paresis ") :  Question  of  liability. 


Case  84.  Lester  Smith  was  a  salesman,  31  years  of  age, 
who,  while  on  a  business  trip,  accompanied  by  his  wife, 
suddenly  developed  grandiose  ideas.  He  originated  a  scheme 
of  cornering  the  phonograph  market.  His  prospects  seemed 
so  certain  to  him,  that  he  hired  an  expensive  suite  of  rooms 
in  a  hotel  at  something  over  $35  a  day.  As  at  the  first 
presentation  of  his  bill  it  was  found  that  he  had  no  money 
to  meet  these  charges,  he  was  taken  into  custody  and  at 
once  transferred  to  a  hospital  for  the  insane,  where  it  was 
discovered  that  he  was  suffering  from  GENERAL  PARESIS. 

I.  What  is  the  patient's  responsibility  for  these  debts? 
Legally  the  patient  or  his  estate  is  responsible  for 
debts  accruing  from  services  rendered  or  goods  re- 
ceived. As  he  is  adjudged  non  compos  mentis  con- 
tracts entered  into  would  not  hold,  and  he  would  not 
be  considered  liable  for  criminal  acts. 

Note:  This  case  shows  how  dangerous  paresis  may  be 
not  only  to  the  life  and  usefulness  of  a  patient,  but  further 
how  it  may  ruin  a  family  financially.  Mr.  Smith's  little 
escapade  used  up  all  the  money  that  he  had  been  able  to 
save  in  his  life  and  when  he  was  taken  to  a  hospital  his  wife 
was  left  destitute. 


296  MEDICOLEGAL  AND   SOCIAL 


Suicidal  attempt  (?)  by  a  neurosyphilitic. 


Case  85.  At  first  Mrs.  Annie  Monks,  a  widowed  seam- 
stress, 50  years  of  age,  did  not  particularly  suggest  syphilis. 
Mrs.  Monks  was  sent  to  us  from  a  general  hospital.  She 
had  been  found  unconscious  in  her  room,  with  gas  turned 
on,  and  a  diagnosis  of  gas  poisoning  was  made.  Mrs.  Monks 
remained  unconscious  for  24  hours,  and  her  apparent  sui- 
cidal attempt  seemed  to  warrant  her  being  sent  to  the 
Psychopathic  Hospital.  Mrs.  M.,  however,  scoffed  at  the 
idea  of  any  attempt  at  suicide,  and  claimed  to  have  had  no 
recollection  of  any  such  affair.  On  the  contrary,  she  had 
gone  to  mass  the  morning  of  the  day  on  which  she  was 
taken  to  the  hospital,  remembered  well  enough  returning 
to  her  room  but  nothing  of  what  followed  until  she  woke  up. 

Mrs.  Monks  was  not  cooperative  and  would  reveal  few 
facts  about  her  history.  For  years,  she  had  had  edema  of 
the  feet  and  palpitation  of  the  heart  (the  heart  was  some- 
what enlarged,  with  a  double  murmur  in  the  aortic  area, 
systolic  louder,  and  a  blood  pressure  of  160  systolic  and 
85  diastolic;  clubbed  fingers;  palpable  liver).  She  had 
been  treated  in  the  out-patient  department  of  a  general 
hospital  for  a  number  of  months.  We  could  obtain  no 
evidence  of  mental  impairment,  particularly  none  of  memory. 

Aside  from  the  heart  lesions  above  indicated,  the  patient 
was  fairly  well  nourished,  with  a  slight  enlargement  of 
superficial  glands,  and  was  otherwise  normal. 

Neurologically,  the  slightly  irregular  pupils  reacted  poorly 
to  light;  the  right  knee-jerk  could  not  be  obtained,  whereas 
the  left  knee-jerk  was  very  active.  Systematic  examination 
revealed  no  other  disorder  except  that  the  abdominal  re- 
flexes could  not  be  obtained. 

Here  we  have,  in  a  cardiac  patient,  a  possibly  or  probably 
accidental  gas  poisoning,  and  little  to  go  upon  for  a  pro- 
founder  diagnosis  than  the  sluggish  irregular  pupils  and 
unilateral  absence  of  knee-jerk. 


MEDICOLEGAL  AND   SOCIAL  2Q7 

The  routine  serum  W.  R.  came  through  as  positive.  Fol- 
lowing custom,  we  examined  the  spinal  fluid,  finding  the 
W.  R.  here  again  to  be  moderately  positive  (strongly  posi- 
tive to  I  cc.,  moderately  to  0.7  cc.,  and  negative  to  0.5,  0.3, 
and  o.l  cc.).  The  gold  sol  index  was  1221000000, 
which  must  be  interpreted  as  syphilitic.  There  were  16 
cells  to  the  cmm.,  the  albumin  was  i  +  ,  and  the  globulin  stood 
at  2+. 

Here,  then,  we  seem  to  have  evidence  of  an  inflammatory 
process  of  the  central  nervous  system,  and  it  is  natural 
forthwith  to  be  sceptical  as  to  the  accidental  nature  of  the 
gas  poisoning.  Perhaps  there  was  an  attempt  at  suicide 
based  upon  a  passing  impulse,  or  perhaps  there  was  a  period 
of  confusion  in  which  the  cock  was  not  turned  off. 

In  any  event,  we  feel  justified  in  making  the  diagnosis  of 
cerebrospinal  syphilis  on  the  basis  of  the  neurological  and 
laboratory  findings.  On  the  whole,  we  are  inclined  to 
make  a  diagnosis  of  VASCULAR  NEUROSYPHILIS  with  a  mod- 
erate involvement  of  the  MENINGES. 

I.  What  is  the  outcome  in  such  cases  as  that  of  Annie 
Monks?  The  case  somewhat  resembled  that  of  Martha 
Bartlett,  who  still  survives.  The  case  of  Annie  Monks 
illustrates  another  outcome.  A  few  days  after  her 
admission,  she  became  unconscious  once  more,  and 
upon  recovery  remained  very  much  confused  and 
aphasic,  moaning,  and  unable  to  handle  herself  well, 
although  without  definite  paralysis.  Three  weeks 
later  the  patient  died,  although  in  the  meantime 
strenuous  antisyphilitic  therapy  was  practised.  Death 
was  sudden.  We  thought  death  due  to  cerebral 
embolism. 


298  MEDICOLEGAL   AND   SOCIAL 


Early  delinquency 

and  neurosyphilis 

in  a  juvenile. 

Case  86.  Frank  Johnson  was  21  years  of  age  when  he 
was  taken  up  by  the  police  for  threatening  his  sister  with 
a  revolver.  The  police  thought  he  deserved  an  examina- 
tion at  the  Psychopathic  Hospital.  The  patient  protested 
that  he  had  threatened  his  sister  only  to  frighten  her  be- 
cause, he  said,  she  nagged  him  and  made  him  nervous.  In 
fact,  they  had  always  had  trouble  as  she  had  'always  nagged 
him  and  they  had  always  fought  together.  Moreover,  their 
mother  always  took  the  sister's  part.  They  had  been  troub- 
ling him  for  days,  and  at  last  Frank  could  stand  it  no  longer. 
His  sister  had  complained  of  the  way  he  treated  her  dog. 
Moreover,  Frank  said  he  had  not  been  feeling  well;  there 
had  been  some  trouble  with  his  stomach;  and  after  one  of 
the  nagging  attacks,  he  had  taken  out  an  old  empty  pistol 
to  scare  his  mother  and  sister. 

In  these  cases,  it  is  good  practice  to  consult  the  sister  also. 
She  said  that  Frank  had  always  been  very  difficult  to  man- 
age, unwilling  to  work,  preferring  to  loaf  about,  spending 
every  obtainable  cent;  he  was  once  in  a  reformatory  for 
several  years,  but  not  reformed  thereby;  recently  given  to 
drinking;  at  times  acting  somewhat  peculiarly  (sitting  at 
the  window  with  his  hat  on,  refusing  to  move). 

Further  mental  examination  of  Frank  showed  that  he  was 
properly  oriented  and  in  possession  of  a  good  memory,  al- 
though he  was  quite  obviously  a  liar.  He  lay  about  in  bed  at 
the  hospital,  saying  that  he  was  too  weak  to  be  up.  He  was 
a  bit  dull,  at  times  not  readily  grasping  ordinary  questions. 

Physically,  Johnson  was  rather  thin;  the  teeth  were  some- 
what peg-shaped  although  far  from  typically  Hutchinsonian. 
The  pupils  were  unequal  and  irregular,  and  failed  to  react 
to  light  or  even  to  accommodation  when  tested.  The  deep 
reflexes  of  arms  and  legs  could  not  be  obtained,  though  the 
superficial  reflexes  were  present.  For  the  rest  systematic 
examination  proved  negative.  Serum  W.  R.  negative. 


MEDICOLEGAL   AND   SOCIAL  299 

The  first  thought  in  such  a  case  would  be  that  the  crimi- 
nological  diagnosis  of  delinquency  would  be  sufficient.  How- 
ever, the  pupillary  disorder  and  the  areflexia  are  suggestive 
despite  the  negative  serum  W.  R.  Resort  was  naturally  had 
to  lumbar  puncture,  whereupon  a  positive  W.  R.  was  found, 
a  characteristically  "  paretic  "  gold  sol  reaction,  globulin,  ex- 
cess albumin,  and  134  cells  per  cmm.  In  short,  it  would  ap- 
pear that  we  must  consider  a  diagnosis  of  JUVENILE  PARESIS, 
and,  in  point  of  fact,  the  patient  deteriorated  rapidly  from 
this  time,  becoming  demented  at  the  end  of  a  few  months. 

1.  How  far  are  the  early  difficulties  of  management  (lead- 

ing to  a  reformatory)  due  to  syphilis?  We  should  not 
dogmatically  say  that  there  is  a  relation  between  the 
early  delinquency  and  syphilis.  Still,  it  is  not  unusual 
to  find  emotional  disorder  and  instability  as  well  as 
delinquency  in  congenital  syphilitics. 

2.  What  suggestion,  if  any,  should  be  made  to  the  patient's 

intelligent  and  seemingly  normal  sister,  two  years 
older?  We  prevailed  upon  Miss  Johnson  to  submit 
to  the  W.  R.  of  the  serum,  which  was  found,  as  in  the 
case  of  Frank,  to  be  negative.  Frank's  sister  should 
undoubtedly  submit  to  a  lumbar  puncture;  but  in 
the  present  phase  of  mental  hygiene,  she  would  be 
difficult  to  persuade. 

3.  How  is  it  possible  to  find  such  a  marked  evidence  of 

congenital  syphilis  in  a  younger  sibling  with  no  evi- 
dence of  syphilis  in  the  elder?  In  the  first  place,  there 
may  be  a  history  of  entrance  of  syphilis  into  the  lives 
of  the  parents  between  the  pregnancies.  However,  in 
other  instances,  there  is  no  evidence  of  such  inter- 
current  syphilis,  and  contrary  to  the  prevailing  opinion 
it  is  not  so  infrequent  to  find  congenital  syphilis  in 
the  younger  brother  or  sister  of  a  normal  person. 

4.  What  can  be  said  of  treatment  in  such  cases?     In  the 

first  place  it  is  clear  that  delinquent  cases  should  be 
tested  far  earlier  for  the  possibility  of  syphilis.  Had 
this  case  been  examined  by  a  neurologist  or  alienist 
many  years  earlier,  it  is  probable  that  the  same  pupil- 
lary signs  and  the  peg-shaped  teeth  would  have  been 
found,  and  that  the  hypothesis  of  syphilis  might  have 
been  raised.  There  is  no  good  evidence  as  yet  that 
these  cases  can  be  markedly  benefited  by  treatment. 


300  MEDICOLEGAL   AND   SOCIAL 


Neurosyphilis  in  a  "  defective  delinquent." 


Case  87.  Vivian  Walker,  22  years  of  age,  was  arrested 
on  [the  streets  of  Boston  for  drunkenness.  Upon  arrival  at 
the  jail,  she  developed  a  series  of  convulsions,  each  lasting 
a  very  brief  time,  with  loss  of  consciousness,  frothing  at  the 
mouth,  and  jerky  movements  of  the  arms  and  legs. 

The  Walker  family  was  known  to  the  police,  since  there 
were  police  records  in  two  generations  on  the  maternal  side. 
The  father  was  regarded  as  of  rather  low-grade  mentality; 
a  sister  had  committed  suicide.  Vivian  herself  had  been 
irregular  at  school,  was  regarded  as  vicious,  and  had  been 
hysterical.  She  had  been  committed  to  a  reformatory  at 
the  age  of  15  years.  In  the  reformatory  she  had  a  number 
of  excited  outbreaks,  with  resentment  of  discipline,  and  these 
outbreaks  presented  hysterical  traits.  After  each  outbreak 
Vivian  was  depressed.  It  was  during  her  stay  at  the  re- 
formatory that  her  sister  committed  suicide.  Vivian  at- 
.tended  the  funeral,  and  the  idea  of  suicide  appears  to  have 
taken  hold  of  her  mind,  as  she  constantly  spoke  of  suicide, 
threatened  suicide,  and  made  several  attempts.  She  claimed 
at  this  time  to  see  visions  and  to  hear  her  sister's  voice. 
On  that  ground  she  had  been  committed  to  a  hospital  for 
the  insane  at  16. 

At  the  hospital  there  were  many  fluctuations  in  mental 
condition.  Vivian  professed  discouragement  on  account  of 
poor  home  influences,  telling  how  her  mother  had  often  been 
in  prison,  allowing  Vivian  to  come  under  the  influence  of 
bad  girls.  Now  and  then  Vivian  had  outbreaks  of  pro- 
fanity and  glass-breaking,  and  she  also  made  at  the  hospital 
for  the  insane  several  half-hearted  attempts  at  suicide.  At 
the  age  of  19  she  was  returned  to  the  reformatory,  whence 
she  was  placed  out  on  probation  and  allowed  to  return  home. 

However,  she  was  shortly  re-committed  to  the  insane 
hospital  in  a  phase  of  excitement,  talking  continuously  of 
men  and  sex  relations,  and  also  of  imaginary  illicit  sex  re- 


MEDICOLEGAL  AND   SOCIAL  3OI 

lations  with  any  man  whom  she  happened  to  see.  Again 
from  time  to  time  she  made  attempts  at  suicide.  However, 
she  was  allowed  to  go  out  on  visit,  returned  to  her  habits, 
and  at  the  time  of  her  arrest  was  living  as  a  prostitute. 

After  her  convulsions  in  jail,  she  was  admitted  to  the 
Psychopathic  Hospital.  At  first  obstinate  and  stubborn, 
later  she  became  tractable.  Special  mental  tests  left  her 
in  the  subnormal  class,  but  we  could  hardly  class  her  as 
feebleminded.  We  were  able  to  observe  her  in  a  number 
of  seizures,  during  which  she  would  drop  to  the  floor,  ap- 
parently lose  consciousness,  writhe  about,  and  assume  the 
position  of  opisthotonos,  the  whole  attack  lasting  but  a 
minute  or  two. 

There  was  pelvic  tenderness,  with  gonococci  in  the  urethral 
smear.  Salpingectomy  had  to  be  performed,  but  after  the 
operation  Vivian  insisted  upon  getting  up  and  running  about 
on  the  second  day,  tearing  the  bandages  from  her  abdomen, 
and  infecting  the  wound.  Outbreaks  of  excitement  also 
followed  the  operation. 

In  the  diagnosis  of  this  case,  we  must  probably  separate 
the  convulsive  phase  from  the  remainder  of  the  phenomena. 
The  conduct  disturbance,  emotional  outbreaks,  and  suicidal 
attempts  date  from  early  youth,  and  no  doubt  the  diagnosis 
defective  delinquent  would  fit  Vivian  from  the  beginning. 
The  hereditary  taint  is  characteristic  enough.  The  sundry 
phenomena  in  the  insane  hospital,  and  particularly  the  hal- 
lucinations, lead  one  to  wonder  whether  Vivian  is  not  pos- 
sibly even  suffering  from  dementia  praecox. 

As  to  the  convulsions,  it  would  hardly  appear  that  they 
are  typically  epileptic,  although  certainly  epileptoid.  Their 
onset  at  22  is  somewhat  unusual.  Several  features  of  the 
seizures  together  with  the  opisthotonos  and  the  previous 
history  of  hysteria,  lead  one  to  think  of  making  the  diag- 
nosis hysteria. 

I.  Can  cerebrospinal  syphilis  cause  the  symptoms?  ^  We 
found  the  serum  W.  R.  to  be  positive  though  Vivian 
denied  syphilitic  infection.  (She  also  denied  gonor- 
rhceal  infection  despite  the  clinical  and  laboratory 
findings.)  We  found  that  the  spinal  fluid  yielded  a 


302  MEDICOLEGAL   AND   SOCIAL 

gold  reaction  of  a  typical  syphilitic  nature,  showed  an 
excess  of  albumin,  a  slight  amount  of  globulin,  and 
130  cells  per  cmm.  Even  these  findings,  however, 
would  perhaps  not  justify  stating  that  the  convulsive 
seizures  are  of  syphilitic  nature.  The  seizures  dis- 
appeared under  the  administration  of  antisyphilitic 
remedies.  It  would  seem,  therefore,  that  the  seizures 
should  be  regarded  as  of  syphilitic  nature.  In  any 
event,  the  diagnosis  of  cerebrospinal  syphilis  is  justifi- 
able. This  syphilis,  however,  is  of  an  active  nature 
and  probably  of  recent  production.  We  should  be  at 
a  loss  to  explain  the  earlier  mental  features  in  Vivian 
as  syphilitic  and  are  therefore  fain  to  associate  the  two 
psychoses,  PSYCHOPATHIC  PERSONALITY  and  DIFFUSE 
CEREBROSPINAL  SYPHILIS. 


MEDICOLEGAL   AND   SOCIAL  303 


NEUROSYPHILIS  ("  paresis  sine  paresi »)  in  an 
habitual  criminal,  a  forger. 


Case  88.*  was  brought  to  the  hospital  by  the 

police.  He  was  charged  with  having  forged  a  check,  and  on 
account  of  the  crudeness  of  the  work  his  mental  condition 
was  suspected. 

Family  History.  The  paternal  grandfather  was  considered 
fast,  drank  a  great  deal  and  was  said  to  be  a  thief.  The 
father  is  said  to  have  been  forced  to  leave  the  State  when  a 
young  man  in  order  to  avoid  the  reformatory.  Paternal 
cousin  murdered  a  man ;  the  sisters  of  this  cousin  said  to  have 
been  wild  and  one  brother  married  a  prostitute.  Nothing 
known  of  maternal  relatives. 

Past  History.  Medical  history  is  unimportant.  He  denies 
syphilis.  His  early  childhood  is  of  little  significance.  He 
was  somewhat  dull  in  school.  At  about  the  age  of  twelve  he 
began  to  lie  and  steal,  and  has  continued  this  ever  since. 
His  attempts  have  all  been  very  crude,  it  is  said,  and  when 
confronted  he  would  strenuously  deny  his  deeds,  even  when 
the  evidence  was  overwhelming.  He  forged  checks,  borrowed 
money  from  all  his  friends,  and  charged  things  at  stores  to 
the  family.  The  family  paid  the  bills  for  a  time,  and  then 
later  had  him  sent  to  a  reform  school.  He  was  married  at 
nineteen,  but  wife  has  left  him  and  obtained  a  divorce.  He 
has  been  excessively  alcoholic  for  years,  and  is  suspected  also 
of  taking  drugs.  He  was  discharged  from  the  navy  dishon- 
orably. He  later  joined  the  army  and  was  discharged  there- 
from on  account  of  "  rheumatism,"  according  to  his  account, 
but  in  reality  deserted.  He  had  finished  a  jail  sentence  of 
thirteen  months  for  forgery  a  little  over  a  year  before  entrance. 

Physical  examination  shows  a  well-developed  and  nour- 
ished man.  The  general  physical  examination  is  negative. 

*  Reprinted  from  article  by  Southard  and  Solomon :  "  Latent 
Neurosyphilis,  the  Question  of  Paresis  sine  paresi"  Boston 
Medical  and  Surgical  Journal,  XXIV,  I. 


304  MEDICOLEGAL  AND  SOCIAL 

The  lungs  show  nothing  abnormal.  The  heart  is  not  enlarged, 
there  are  no  murmurs  or  irregularities;  blood  pressure,  145 
systolic.  The  alimentary  system  is  negative.  No  palpable 
lymph  glands.  Neurological  examination:  pupils  equal  and 
react  to  light  and  accommodation.  Extraocular  movements 
well  performed.  Tongue  projects  in  the  median  line,  with  no 
tremor.  There  is  no  evidence  of  facial  paresis  or  weakness 
of  the  muscles.  The  biceps,  triceps,  knee-jerks  and  ankle- 
jerks  are  present  and  equal  on  the  two  sides.  There  is  no 
Gordon,  Babinski  or  Oppenheim;  no  ankle  clonus.  There  is 
no  tremor  of  the  extended  hands.  No  Romberg  sign.  There 
is  a  little  difficulty  in  the  finger- to- finger  test.  There  is  no 
sensory  disturbance  either  subjective  or  objective.  No  ten- 
derness over  nerve  trunks. 

Mental  examination  shows  nothing  of  a  psychotic  nature. 
Patient  is  well  oriented;  memory  for  remote  and  recent 
events  is  well  preserved,  school  knowledge  well  retained,  grasp 
on  current  events  good ;  no  delusions  or  hallucinations  elicited. 
Patient  is  not  feeble-minded,  according  to  the  intelligence 
tests  of  Binet  and  Simon  and  Yerkes-Bridges,  but  shows  poor 
attention  and  gives  evidence  of  weakness  in  volitional  spheres ; 
is  very  suggestible. 

To  summarize  the  case,  then,  we  have  a  man  of  thirty  years 
of  age  who  has  shown  criminalistic  and  anti-social  tendencies 
since  childhood,  whose  general  physical  and  neurological  ex- 
amination is  negative  (excepting  the  laboratory  tests),  whose 
mental  examination  shows  no  psychotic  symptoms,  and  who 
seems  not  feeble-minded.  In  other  words,  with  the  excep- 
tion of  the  serological  and  chemical  findings  in  the  blood 
and  cerebrospinal  fluid,  there  is  nothing  to  suggest  that  he  is 
more  than  a  "  criminal  type." 

Wassermann  reaction  in  blood  serum  positive. 

Wassermann  reaction  in  cerebrospinal  fluid  positive.  Ex- 
amination of  cerebrospinal  fluid:  globulin ++,  albumin ++, 
cells  55  per  cubic  millimeter;  large  lymphocytes,  9.1  per 
cent;  small  lymphocytes,  90  per  cent;  plasma,  90  per  cent. 
Gold  sol  reaction,  3321000000. 


MEDICOLEGAL  AND  SOCIAL  305 

Can  the  criminalistic  tendencies  be  condoned  in  this 
case  on  the  ground  of  neurosyphilis?  As  a  matter  of 
fact  the  delinquencies  in  this  patient  reach  back  to 
early  childhood  and  as  there  is  no  evidence  of  con- 
genital syphilis  it  cannot  be  held  that  syphilis  had  any 
bearing  in  the  causation  of  symptoms.  Even  were  the 
delinquencies  only  of  recent  date  it  is  doubtful  if  the 
court  would  take  cognizance  of  the  laboratory  findings 
in  the  absence  of  definite  mental  symptoms.  In  this 
connection  it  may  be  stated  that  the  court  takes  cog- 
nizance only  of  the  acts  of  a  patient  at  time  of  exami- 
nation, and  not  of  the  history  or  laboratory  findings, 
in  committing  a  person.  We  have  had  several  patients 
who  from  history,  physical  signs  and  laboratory  tests 
made  the  diagnosis  of  paretic  neurosyphilis  easy  and 
yet  who  could  not  be  committed  because  they  were 
mentally  clear  at  the  time.  Such  patients  may  be  of 
grave  potential  danger  to  themselves  and  families,  and 
present  numerous  social  problems.  See  case  of  Joseph 
Wilson  (95). 


306  MEDICOLEGAL   AND   SOCIAL 


JUVENILE  PARETIC  NEUROSYPHILIS  ("  juve- 
nile paresis  ")  with  initial  trauma. 


Case  89.  Margaret  Tennyson  was  a  small  girl  of  six 
years,  described  as  having  been  normal  until  run  down  by  a 
double-runner  sled  about  13  months  before  her  arrival  at  the 
hospital.  The  change  was  stated  to  be  remarkable.  "  She 
was  as  unlike  her  own  self  as  darkness  and  daylight."  Once 
fat  and  sunny,  talkative  and  demonstrative  with  her  toys,  now 
Margaret  had-become  silent,  sullen,  worried,  and  of  a  violent 
temper,  stubborn  and  unmanageable.  It  does  not  appear 
that  the  patient  was  seriously  injured  by  the  double-runner, 
as  she  was  able  to  walk  a  short  distance  home.  Shortly, 
however,  she  began  to  have  trouble  with  her  feet  (diagnosed 
at  the  time  as  flat-foot),  and  thereafter  her  whole  character 
and  disposition  changed.  Upon  arrival  at  the  hospital,  the 
patient  walked  with  a  typical  scissors  gait  of  spastic  para- 
plegia. 

Physical  examination  was  very  difficult  through  lack  of 
cooperation  and  a  screaming  and  kicking  resistance  upon 
every  attempt.  There  was  a  suggestion  of  hydrocephalus 
in  the  protrusion  of  the  forehead.  The  pupils  reacted  readily 
to  light  and  accommodation.  The  knee-jerks  were  active, 
but  there  was  otherwise  no  disorder  of  reflexes.  The  patient 
had  great  difficulty  in  getting  up  from  the  floor,  and  for  the 
most  part  insisted  upon  lying  in  ventral  decubitus  on  the  floor, 
crying  when  attempt  was  made  to  raise  her.  An  attempt 
was  made  to  test  her  by  the  Binet  scale,  by  which  she  was 
found  to  rate  at  2-f  years  although  a  portion  of  this  low- 
rating  was  thought  to  be  due  to  a  failure  of  cooperation. 

The  family  history  threw  little  or  no  light  upon  the  case. 
The  parents  were  living  and  well;  a  brother  of  16  years  was 
at  work  in  the  market  district;  two  of  the  other  siblings  are 
in  the  first  and  second  grades  at  school  and  regarded  as  ex- 
ceptionally bright  by  their  teachers.  The  fourth  was  the 
patient,  Margaret;  a  fifth  had  died  at  9  weeks  of  heart  trouble; 


Juvenile  paresis  —  spastic  paraplegia.     5  years. 


MEDICOLEGAL   AND    SOCIAL  307 

the  sixth,  seventh,  and  eighth,  of  3,  i^  years  and  3  months 
respectively,  appeared  entirely  well.  There  were  no  mis- 
carriages or  stillbirths. 

The  scissors  gait  and  spasticity  seem  to  point  undoubtedly 
to  organic  disease  of  the  nervous  system,  along  with  which 
the  mental  deterioration  seemed  to  suggest  an  active  pro- 
gressive involvement  of  the  cerebrum.  The  history  seemed 
to  be  convincing  that  the  child  was  not  an  instance  of  con- 
genital feeblemindedness. 

A  neurologist's  clinical  diagnosis  would  naturally  be  syphilis. 
In  point  of  fact,  this  diagnosis  was  borne  out  by  the  laboratory 
tests,  which  showed  a  positive  W.  R.  in  the  serum  and  spinal 
fluid,  positive  globulin,  a  slight  excess  of  albumin,  and  a 
syphilitic  gold  sol  reaction. 

I.  What  is  the  significance  of  the  trauma  in  the  case  of 
Margaret  Tennyson?  The  trauma  seemed  to  the 
family  the  precipitating  cause.  We  find  cases  of  gen- 
eral paresis  in  adults  very  definitely  following  trauma, 
yet  neurosyphilis,  both  in  adults  and  in  younger  patients, 
mainly  occurs  without  trauma.  On  the  whole,  in  this 
case,  it  is  perhaps  safer  to  regard  the  trauma  as  mere 
coincidence.  A  sister  older  than  Margaret  was  found 
upon  examination  to  have  a  positive  W.  R.  The  other 
children  could  not  be  examined. 


308  MEDICOLEGAL   AND    SOCIAL 


Traumatic  form  of  PARETIC  NEUROSYPHTLIS 
("general  paresis"). 


Case  90.  The  point  about  Joseph  O'Hearn  was  his  entire 
mental  soundness  up  to  the  time  of  an  injury  at  work,  when 
he  was  blown  through  a  double  window  in  an  explosion, 
badly  bruising  his  head.  Shortly  after  the  accident,  although 
not  immediately,  the  patient  began  to  show  signs  of  mental 
disorder,  doing  very  foolish  things,  losing  his  memory,  and 
becoming  unable  to  work. 

It  was  eight  months  after  the  explosion  when  O'Hearn,  at 
the  age  of  36,  was  admitted  to  the  hospital  with  general  mental 
impairment.  O'Hearn  was  confused  and  disoriented  for 
time  and  place,  although  he  seemed  to  understand  that  he 
was  in  a  hospital.  He  was  given  to  foolish  laughter  and  a 
silly  manner.  There  was  considerable  emotional  disorder; 
judgment  was  clearly  impaired,  and  memory  was  poor. 

Physically,  there  was  little  to  be  found  except  upon  neuro- 
logical examination.  The  right  knee-jerk  was  greater  than 
the  left;  the  tongue  and  fingers  showed  marked  tremor,  there 
was  a  speech  defect  and  writing  disorder. 

On  the  whole,  it  seemed  impossible  not  to  make  the  diag- 
nosis GENERAL  PARESIS,  especially  in  view  of  the  laboratory 
tests,  with  positive  W.  R.  in  both  serum  and  fluid,  a  "  pa- 
retic  "  type  of  gold  reaction,  59  cells  per  cmm.,  excess  albumin, 
and  a  large  amount  of  globulin. 

i.  What  is  the  relation  of  the  trauma  to  the  paresis? 
Trauma  is  regarded  as  a  precipitating  cause,  and  In- 
dustrial Accident  Commissions  have  been  known  to 
allow  damages  in  such  cases.  Mott  believes  that  the 
symptoms  of  a  post-traumatic  paresis  must  not  develop 
until  after  a  week's  interval  of  freedom  from  symptoms, 
since  he  believes  that  time  is  required  to  destroy  or 
irritate  the  brain  to  the  point  of  producing  the  paretic 
picture.  Our  data  are  in  agreement  with  those  of 
Mott.  Mott  also  points  out  that  gumma  sometimes 
occurs  at  the  site  of  the  trauma. 


MEDICOLEGAL  AND   SOCIAL  309 


False    claim   for   compensation   in   neurosyphilis. 


Case  91.  The  facts  in  the  case  of  Levi  Sussman  can  be 
brought  out  by  the  following  extracts  from  a  report  to  the 
Industrial  Board:  A  claim  was  made  to  the  Board  that  the 
symptoms  had  developed  after  a  fall  from  a  building,  some 
nine  months  before  hospital  observation.  No  connection  could 
be  found  between  this  accident  and  the  PARETIC  NEURO- 
SYPHILIS found.  We  introduce  the  case  to  emphasize  the 
possibility  that  irrelevant  accidents  may  be  regarded  by 
ignorant  or  unscrupulous  persons  as  setting  up  a  mental 
disorder  for  which  damages  are  claimed.  If  symptoms  are 
already  in  existence  before  the  accident  and  are  not  especially 
increased  thereafter,  naturally  no  damages  should  be  re- 
covered. Unscrupulous  persons  may  falsify  about  the  pre- 
traumatic  history  and  claim  the  development  of  symptoms 
immediately  after  the  accident.  Such  claims  are  beyond 
question  to  be  viewed  with  the  greatest  suspicion.  Some 
days  or  weeks  should  elapse  before  definite  symptoms  in 
post-traumatic  paresis  appear.  Just  how  long  an  interval 
may  elapse  between  trauma  and  paretic  symptoms  and  shall 
entitle  the  case  to  be  regarded  as  one  of  traumatic  paresis, 
is  perhaps  a  matter  of  doubt.  It  would  seem,  however,  on 
general  grounds  that  three  months  is  the  longest  period  in 
which  the  post-traumatic  effects  are  likely  to  be  delayed. 

The  question  of  traumatic  paresis  is  of  great  interest  on 
account  of  the  war.  The  great  strain  under  which  the  men 
at  the  front  live  and  the  physical  injury  due  to  being 
"  buried  "  is  probably  responsible  for  an  increasing  number 
of  cases  of  neurosyphilis.  Such  at  least  is  the  impression 
of  Canadian  medical  officers  with  whom  we  have  spoken. 
See  Section  VI,  Neurosyphilis  and  the  War. 


3IO  MEDICOLEGAL   AND    SOCIAL 


Traumatic  exacerbation  (?)  in  PARETIC  NEURO- 
SYPHILIS  ("  general  paresis  "). 


Case  92.  The  case  of  Joseph  Larkin  was  of  note  from  the 
point  of  view  of  the  Industrial  Accident  Board.  This  Irish 
teamster  was  said  to  have  been  injured  in  his  head  two  or 
three  months  before  coming  up  for  examination  at  the  age  of 
45.  For  a  week  Larkin  had  had  frontal  headaches,  had  been 
sleeping  poorly,  and  had  been  somewhat  worried.  In  fact, 
he  had  stopped  work.  The  W.  R.  of  the  serum  was  positive 
and  a  diagnosis  of  PARESIS  could  be  made.  The  case  did  not 
come  up  for  consideration  by  the  Industrial  Board  until  two 
years  after  his  initial  appearance. 

The  physical  examination  showed  irregular  pupils,  sluggish 
pupillary  reactions,  Achilles  absent,  swaying  in  the  Romberg 
position,  enlargement  of  the  heart  to  the  left,  positive  W.  R. 
of  the  blood  and  of  the  spinal  fluid. 

Mentally,  the  patient's  orientation  for  place  was  poor 
and  his  memory  defective.  Emotionally  he  was  depressed  or 
apathetic  and  was  apprehensive.  His  flow  of  thought  was 
slow,  and  his  insight  into  his  condition  poor. 

It  is  interesting  that  a  variety  of  causes  have  been  assigned 
in  this  case  for  the  condition:  such  as,  his  work,  anemia, 
unhygienic  surroundings,  and  arteriosclerosis. 

This  case  is  not  a  sharply-defined  case  of  post-traumatic 
general  paresis,  since  there  had  undoubtedly  been  a  variety 
of  mental  changes  before  the  accident.  Accordingly,  re- 
covery of  damages  to  a  full  amount  could  hardly  be  expected 
as  in  certain  cases  in  which  the  phenomena  of  paresis  appear 
only  after  the  trauma, 


• 


Post-traumatic  cranial  gumma  —  developing  13  months  after  local  injury  of  skull. 


MEDICOLEGAL  AND   SOCIAL  311 


Trauma :    syphilitic  lesion  of  skull  at  site  of  injury. 


Case  93.  The  medicolegal  interest  of  Richard  Marshall 
is  extreme,  as  may  be  seen  from  the  following  brief  report  by 
the  Psychopathic  Hospital  to  the  Industrial  Board. 

"  As  to  the  case  of  Richard  Marshall,  a  patient  under 
the  provisions  of  the  temporary  care  act  from  December 
i  to  December  10,  inclusive,  this  case  has  proved  un- 
usually interesting  in  that  the  patient  has  proved  to  be 
syphilitic  by  the  Wassermann  reaction  of  the  blood. 
There  is  no  evidence  of  syphilis  in  the  examination  of 
the  cerebrospinal  fluid.  The  X-ray  examination  of  the 
skull,  taken  in  connection  with  the  Wassermann  reac- 
tion of  the  blood,  warrants  the  diagnosis  of  syphilitic 
osteitis  of  the  skull  at  the  site  of  the  old  injury.  We 
regard  his  present  condition  as  shown  by  the  X-ray 
as  a  syphilitic  bone  condition  predisposed  to  by  the 
injury.  We  do  not  find  that  the  patient  has  any  fea- 
tures of  traumatic  neurosis. 

"  Mentally,  having  an  actual  age  of  30,  patient  grades 
at  ii. 2  years.  It  may  be  that  patient  has  always  been 
a  moron.  He  has  earned  about  $8.30  a  week. 

"  We  regard  the  patient  as  deserving  treatment  and 
feel  that  responsible  parties  in  the  case  would  do  well  to 
have  such  treatment  instituted." 

The  principal  symptom  of  which  Marshall  complained 
was  headache  chiefly  felt  in  the  region  of  the  osteitis. 
There  was  marked  sensitiveness  to  percussion  in  this  area. 
It  is  of  course  difficult  to  decide  whether  the  headache 
was  entirely  due  to  the  gummatous  lesions  or  whether  the 
trauma  had  caused  contusions  of  the  brain  as  well.  It  is 
also  possible  that  the  dura  underlying,this  area  was  involved. 


312  MEDICOLEGAL  AND   SOCIAL 


OCCUPATION-NEUROSIS    in    a   granite-cutter: 
SYPHILITIC  NEURITIS? 


Case  94.  David  Fitzpatrick  was  a  case  referred  to  the 
Psychopathic  Hospital  by  the  Industrial  Accident  Board. 
He  was  a  granite-cutter  of  52  years  of  age,  and  had  begun  to 
complain  of  pain  in  the  forearm,  extending  back  from  the 
elbow,  about  six  months  before  admission.  It  seems  that 
the  patient  had  been  growing  progressively  worse  and  had 
thought  he  would  have  to  quit  work  because  of  difficulty  in 
grasping  the  hammer.  A  physician  had  told  him  that  he 
must  stop  his  work  at  granite-cutting  or  else  he  would  en- 
tirely lose  the  use  of  his  arm.  He  was  in  point  of  fact  laid 
off  because  of  slackness  of  work  and  had  been  unable  to  get 
work  again.  The  pain  in  the  arm,  however,  had  continued 
and  at  times  was  very  severe.  Sometimes  the  pain  and  the 
worry  led  to  insomnia.  Fitzpatrick  wanted  the  insurance 
company  to  pay  certain  accumulated  bills,  and  maintained 
that  he  would  be  able  to  do  work  at  $15  a  week  if  work  could 
be  found  for  him.  The  general  situation  in  this  case  can  be 
gathered  from  the  following  abstract  from  the  report  to  the 
Industrial  Accident  Board. 

"  Secretary  Industrial  Accident  Board, 
"Dear  Sir: 

"  In  re  David  Fitzpatrick 

referred  to  us  with  a  copy  of  an  impartial  report  filed 
by  the  Massachusetts  General  Hospital,  —  we  concur 
with  said  impartial  report  that  there  is  now  no  evidence 
of  paralysis  of  the  arm.  We  do  not  find  that  the 
positive  Wassermann  reaction,  although  it  indicates  a 
history  of  syphilis,  has  affected  the  patient  other  than 
possibly  to  have  reduced  his  general  mental  capacity. 
Our  special  tests  yielded  a  percentage  of  62%  of  what 
a  patient  of  his  age  and  station  should  possess.  There 
seems,  however,  to  be  no  connection  between  this  reduc- 
tion of  mental  capacity  and  the  difficulty  with  the  arm. 
We  cannot  connect  the  history  of  alcoholism  with  the 
arm  trouble. 


MEDICOLEGAL  AND   SOCIAL  313 

"There  is  some  evidence  that  other  stone  workers 
have  at  times  shown  such  effects. 

l<  The  patient's  fairly  circumstantial  account  of  his 
difficulty  seems  to  point  to  a  degree  of  myalgia  or  mus- 
cular pain  in  the  region  of  the  forearm  when  held  in  a 
certain  position  and  a  feeling  of  numbness  in  the  third 
and  fourth  fingers.  Whether  these  phenomena  are  due 
to  local  pressure  upon  nerves  in  the  upper  part  of  the 
forearm  due  to  neuritis,  or  whether  we  are  dealing  with 
a  functional  neuralgic  phenomenon  is  a  question. 

"  We  have  applied  some  special  tests  for  faradic 
sensibility  to  all  the  fingers  of  both  hands  and  have 
found  that  the  fingers  of  the  right  hand  are  still  less 
sensitive  than  those  of  the  left,  particularly  the  thumb 
and  the  little  finger.  This  test  has  not  yet  been  ap- 
plied in  a  sufficiently  large  number  of  cases  to  prove 
any  difficult  point,  nevertheless  the  findings  are  in  line 
with  the  patient's  own  circumstantial  account  of  former 
feelings  of  numbness  in  the  third  and  fourth  fingers  of 
[the  right  hand. 

"  Obviously,  then,  our  opinion  is  that  there  is  still  to 
be  found  some  effect  of  the  disease,  whatever  it  was, 
which  caused  the  patient  to  knock  off  work.  If  we  had 
more  experience  with  such  cases  and  more  data  with  the 
new  test  which  we  have  applied,  we  should  perhaps  be 
inclined  to  admit  the  diagnosis  of  occupation  neuritis. 
and  to  suppose  structural  alterations  in  the  nerve  trunks 
corresponding  with  the  location  of  the  muscular  pain 
and  the  anesthesia  of  fingers  and  the  dulling  of  electric 
sense,  but  in  the  present  stage  of  our  experience,  it 
is  probably  wiser  to  call  the  case  one  of  occupation 
neurosis." 

It  is  clear  that  the  W.  R.  in  this  case  was  of  peculiar  value 
in  at  least  partially  clearing  up  the  findings,  yet  it  must  be 
remembered  that  it  is  a  principle  of  the  modern  adminis- 
tration of  industrial  accident  boards  and  similar  organizations 
that  it  is  the  employer's  lookout  whether  the  employee  has 
syphilis.  Recovery  can  be  made  as  if  the  injury  were  due 
wholly  to  an  accident.  It  was  not  possible  however  def- 
initely to  prove  or  disprove  a  relation  of  syphilis  in  the  form 
of  a  syphilitic  neuritis  to  the  condition  in  this  case. 

The  special  tests  above  referred  to  are  the  electric  sensory 
threshold  tests  of  E.  G.  Martin. 


314  MEDICOLEGAL   AND   SOCIAL 


Character  change:    neurosyphilis. 


Case  95.  Joseph  Wilson  offered  a  very  serious  social 
problem.  He  was  the  father  of  two  children,  and  his  wife 
was  pregnant  at  the  time  of  his  admission  to  the  Psycho- 
pathic Hospital.  He  was  a  husky-looking  man  of  33  years  of 
age,  but  for  the  past  four  years  he  had  been  deteriorating  in 
his  work;  he  had  been  drinking  heavily,  and  finally  had  stolen 
to  obtain  money  for  liquor.  It  was  on  account  of  his  alco- 
holism and  delinquency,  which  were  taken  as  an  indication  of 
change  of  character,  that  he  was  sent  to  the  hospital. 

Examination  on  his  arrival  disclosed  at  once  that  there  was 
more  to  the  case  than  alcoholism,  for  the  neurological  ex- 
amination showed  that  the  pupils  were  irregular,  the  right 
being  larger  than  the  left,  both  reacting  sluggishly  to  light, 
and  there  was  an  inequality  in  the  reaction  of  the  two  eyes, 
the  left  being  better  than  the  right.  The  tendon  reflexes 
were  exaggerated,  with  ankle  clonus  on  both  sides,  more 
marked  on  the  right.  There  was  also  a  marked  speech  de- 
fect. Otherwise  the  physical  examination  showed  nothing 
of  importance. 

The  W.  R.  of  the  blood  and  spinal  fluid  was  strongly 
positive.  The  globulin  test  was  strongly  positive,  the  albu- 
min was  markedly  increased,  there  were  74  cells  per  cmm., 
and  a  gold  sol  reaction  of  the  "  paretic  "  type. 

A  formal  mental  examination  did  not  show  very  much  of 
consequence;  his  memory  showed  no  marked  impairment, 
he  was  not  deluded  or  hallucinated,  and  he  had  a  pretty 
good  insight  into  his  failings.  However,  he  was  somewhat 
childish,  and  his  irritability  was  quite  marked.  Were  one 
to  rely  upon  the  mental  signs  alone,  it  is  probable  that  a  diag- 
nosis of  chronic  alcoholism  with  deterioration  would  be  made; 
but  in  the  presence  of  the  physical  findings  and  the  laboratory 
tests,  the  diagnosis  of  neurosyphilis  had  to  be  given.  It  is 
obvious  that,  while  the  patient  was  suffering  from  a  pro- 
gressive brain  disease,  and  while  he  did  show  mental  symp- 


MEDICOLEGAL  AND   SOCIAL  315 

toms,  there  was  not  sufficient  ground  on  which  to  commit 
him,  and  therefore  he  had  to  be  turned  out  into  the  com- 
munity. As  a  matter  of  fact,  he  was  not  prosecuted  on  ac- 
count of  his  theft,  because,  although  legally  responsible,  it 
was  felt  that  his  disease  was  at  the  basis  of  the  character 
change  which  had  led  him  into  difficulties.  Further  de- 
velopments of  his  relations  with  society  had  to  be  considered, 
however.  It  was  possible  to  get  him  to  discontinue  the  use 
of  alcohol  altogether,  and  for  nearly  a  year  he  has  taken  no 
alcoholic  liquor  and  has  been  self-supporting.  However,  his 
irritability  has  been  very  great,  making  it  very  difficult  for 
his  wife  to  live  with  him,  and  causing  his  sister  to  break  off 
all  relations  with  him. 

Here,  then,  is  a  man  with  a  marked  CHARACTER  CHANGE 
as  the  result  of  neurosyphilis,  so  that  it  is  difficult  for  him 
to  maintain  the  usual  social  relations.  It  does  not  seem 
possible  to  remove  him  from  the  community. 

i.  May  one  speak  of  general  paresis  without  mental  symp- 
toms? If  one  considers  general  paresis  a  mental  disease, 
of  course  it  cannot  exist  without  mental  symptoms. 
However,  if  one  considers  the  disease  as  a  chronic 
syphilitic  meningoencephalitis  characterized  by  its 
pathological  anatomy,  then  one  may  readily  speak  of 
general  paresis  although  no  real  evidence  of  mental 
symptoms  can  be  discovered.  It  would  seem  that  we 
must  take  this  attitude  with  our  present  conception 
of  brain  localization,  for  it  is  easy  to  conceive  of  a 
general  paretic  process  affecting  areas  which  do  not 
definitely  relate  to  psychic  function.  And  further,  such 
a  process  may  exist  but  not  be  of  such  a  grade  as  to 
cause  mental  symptoms. 


31 6  MEDICOLEGAL  AND   SOCIAL 


The  neurosyphilitic's  family  should  not  be  forgot- 
ten in  diagnosis  and  treatment. 


Case  96.  The  Bornstein  family  is  remarkable.  Let  us 
hang  the  story  on  Becky,  the  mother,  an  Austrian  woman  of 
43  years,  who  appears  to  have  been  perfectly  well  up  to  within 
a  year.  About  a  year  ago,  Mrs.  Bornstein  began  to  suffer 
from  severe  headaches,  which  were  treated  with  apparent 
success  by  an  osteopath:  at  all  events,  Mrs.  Bornstein  re- 
covered therefrom  in  about  six  months.  However,  two 
months  later,  she  had  a  convulsion,  with  foaming  at  the 
mouth,  blueness  of  face,  and  general  muscular  stiffening. 
The  convulsion  lasted  for  several  minutes.  Again,  a  fort- 
night before  admission,  the  patient  had  five  convulsions  of 
an  identical  nature  in  a  single  night. 

Moreover,  since  the  first  convulsion,  Mrs.  Bernstein's 
mental  condition  has  altered  and  become  variable,  so  that  at 
times  she  is  excited,  at  times  depressed.  She  would  assert 
inaccurately  that  there  was  some  one  in  the  house,  and  that 
she  had  at  different  times  committed  crimes  of  a  heinous 
nature.  Now  and  then  she  would  seem  to  see  moving  pic- 
tures. Her  memory  was  poor  and  she  seemed  to  believe 
that  events  of  five  or  six  years  ago  had  just  happened. 

The  pupils  were  sluggish,  the  knee-jerks  and  ankle- jerks 
were  absent,  there  was  slight  ataxia,  and  there  was  speech 
defect.  The  suspicion  of  neurosyphilis  was  so  strong  that  it 
seemed  surprising  that  the  W.  R.  of  the  blood  serum,  even 
after  repeated  tests  and  after  the  provocative  injection  of 
salvarsan,  proved  negative.  However,  the  spinal  fluid  yielded 
a  positive  W.  R.,  and  a  gold  sol  reaction  of  the  "  paretic  " 
type,  together  with  12  cells  per  cmm.,  and  a  marked  increase 
of  albumin,  with  positive  globulin.  It  would  seem  warrant- 
able to  make  a  diagnosis  at  least  of  syphilis  of  the  nervous 
system  in  this  case,  but  it  is  a  question  whether  we  should  be 
warranted  in  making  the  diagnosis  general  paresis. 

That  the  diagnosis  is  doubtful  may  perhaps  be  seen  from 


MEDICOLEGAL  AND   SOCIAL  ^  317 

the  variety  of  diagnoses  in  the  rest  of  the  family.  In  the 
first  place,  Mrs.  Bornstein's  husband  admits  syphilitic  infec- 
tion many  years  before.  He  states  also  that  his  wife  after 
marriage  showed  signs  of  syphilis  and  received  some  treat- 
ment, although  limited.  It  is  stated  also  that  the  husband 
himself  at  this  time  has  a  positive  W.  R.  and  has  stiff  pupils 
and  petit  mal  attacks.  The  oldest  son,  22  years  of  age,  is 
confined  in  an  institution  with  juvenile  paresis.  The  second 
son  has  recently  died  at  the  age  of  20  years,  receiving  a  diag- 
nosis of  rupture  of  the  aorta.  A  third  son,  19  years  of  age, 
has  the  appearance  of  having  achondroplasia,  although  the 
proportions  of  his  limbs  do  not  quite  correspond  with  those 
of  an  achondroplast.  The  fourth  son,  17  years  of  age,  is 
suffering  from  caries  of  the  spine.  A  fifth  son,  14  years  old, 
is  neurotic  and  has  the  so-called  Olympic  forehead.  The 
sixth  and  last  son  died  shortly  after  birth  of  unknown  cause. 


31 8  MEDICOLEGAL   AND    SOCIAL 


Neurosyphilitic's    normal-looking     family     proved 
syphilitic. 


Case  97.  Walter  Heinmas  was  a  draughtsman  33  years  of 
age  when  he  was  brought  to  the  Psychopathic  Hospital  suf- 
fering from  mental  disease.  This  was  diagnosed  as  general 
paresis,  both  on  account  of  the  clinical  symptomatology  and 
on  account  of  the  laboratory  findings.  In  fact,  it  was  a  case 
of  the  classical  type  with  marked  euphoria  and  grandiosity. 

As  is  the  routine  procedure  at  the  Psychopathic  Hospital, 
in  the  case  of  all  syphilitic  patients,  the  family  was.  sent  for. 
This  consisted  of  the  wife  and  two  daughters,  aged  9  and  7 
respectively.  The  patient  denied  any  knowledge  of  a  syphil- 
itic infection.  The  wife,  also,  gave  no  history  of  any  primary, 
secondary,  or  tertiary  symptoms;  there  had  been  no  abor- 
tions, miscarriages,  or  stillbirths;  both  children  had  been 
born  at  term  and  had  been  entirely  healthy.  Examination 
showed  that  the  mother  had  no  signs  referable  to  syphilis,  and 
that  both  the  children  were  mentally  well  endowed,  with 
good  physique  and  showing  no  stigmata  of  congenital  syphilis. 
Still  the  W.  R.  of  all  three  (the  mother  and  the  two  children) 
was  positive  in  the  blood  serum.  These  tests  were  repeated 
several  times  on  the  children,  with  and  without  injections 
of  salvarsan,  and  they  remained  consistently  positive. 

1.  Are  these  children  to  be  considered  congenital  syphilitics 

despite  the  absence  of  stigmata  or  symptoms?  We 
must  consider  these  children  as  congenital  syphilitics 
and  candidates  for  the  group  frequently  spoken  of  as 
syphilitis  hereditaria  tarda. 

2.  What  is  the  frequency  of  syphilitic  involvement  in  the 

mate  and  children  of  paretics?  In  our  series,  we  have 
found  that  about  15%  of  the  marriages  where  one 
member  develops  paresis,  result  in  sterility;  that  in 
1 8%  abortions,  miscarriages  and  -stillbirths  occur; 
and  that  in  15%  positive  W.  R.  is  obtained.  We  have 
adopted  the  motto:  "The  families  of  paretics  are  the 
families  of  syphilitics." 


MEDICOLEGAL   AND    SOCIAL  319 


Neurosyphilis :    question  of  marriage. 


Case  98.  Mr.  Jacobs'  wife  was  admitted  to  the  hospital 
with  a  diagnosis  of  general  paresis.  A  few  weeks  after  her 
admission,  she  died  as  a  result  of  her  disease.  According  to 
our  routine,  her  husband  and  the  children  were  examined  for 
evidences  of  syphilis. 

Mr.  Jacobs'  blood  serum  was  found  on  repeated  tests 
to  be  positive.  He  resolutely  denied  any  knowledge  of  a 
syphilitic  involvement,  but  it  was  later  learned  from  his 
brother  that  about  two  years  before  his  marriage  —  that  is, 
more  than  25  years  before  we  saw  him  —  he  had  acquired 
syphilis  and  had  had  a  very  small  amount  of  treatment. 

Mr.  Jacobs'  was  put  upon  antisyphilitic  treatment  in  the 
form  of  injections  of  .3  gram  of  salvarsan  every  two  weeks 
with  occasional  intramuscular  injections  of  mercury  salicylate. 
After  seven  months  of  treatment,  the  blood  serum  still  re- 
mained positive.  At  about  this  time,  the  patient  came  to  us 
to  ask  about  getting  married  again.  He  said  that  he  was 
living  with  his  sister,  who  kept  telling  him  that  he  was  the 
cause  of  his  wife's  death,  and  this  was  so  unpleasant  that  he 
desired  to  start  a  home  for  himself  again! 

1.  What  advice  should  be  given?     It  is  a  general  opinion 

that  the  longer  the  period  after  the  initial  infection,  the 
less  the  chances  of  infecting  a  partner.  This  chance 
is  further  reduced  under  antisyphilitic  treatment,  of 
which  a  considerable  amount  had  been  given  in  the 
case  of  Mr.  Jacobs.  However,  when  one  considers 
the  trickiness  of  syphilis  and  the  fact  that  there  is  some 
chance  of  infection,  which  we  would  apparently  over- 
look if  we  gave  him  permission  to  marry  at  this  time, 
the  only  possible  course  was  to  tell  the  patient  that  he 
should  not  consider  marriage  until  his  Wassermann 
had  become  negative  and  remained  so  for  some  time. 
The  children  in  this  case  were  negative. 

2.  What  is  the  physician's  duty  to  the  family  of  a  syphilitic 

patient?     It  is  our  firm  conviction  that  it  is  the  duty  of 


32O  MEDICOLEGAL  AND   SOCIAL 

every  physician  to  his  syphilitic  patient,  to  the  patient's 
family,  and  to  the  community,  to  examine  the  mate 
and  the  children  for  evidence  of  syphilis  acquired  or 
congenital  and  to  offer  treatment  if  it  is  found  to  be 
needed.  This  is  one  of  the  chief  means  at  our  disposal 
today  to  prevent  the  late  disasters  of  syphilis,  acquired 
or  congenital,  for  by  such  examinations  the  syphilitic 
condition  is  discovered  before  lesions  have  occurred 
which  are  irreparable.  We  know  that  the  mate  and 
children  of  a  syphilitic  patient  have  been  exposed  to 
syphilitic  involvement,  and  it  is  our  duty  as  physicians 
in  possession  of  such  knowledge,  and  as  guardians  of  the 
public  health,  to  investigate  such  cases,  so  that  if  they 
be  found  to  have  syphilis,  steps  may  be  taken  to  treat 
them  early. 

3.  How  much  danger  is  there  of  causing  unhappiness  and 

breaking  up  families  by  this  procedure?  This  question 
offers  a  chance  for  many  theoretical  answers.  The 
facts  are,  however,  that  in  doing  this  as  a  routine 
for  nearly  three  years  and  examining  several  hundred 
families,  there  has  been  no  instance  to  our  knowledge 
in  which  a  family  has  been  broken  up  or  grave  difficul- 
ties have  been  encountered  by  this  procedure. 

4.  In  what  percentage  are  the  mates  or  children  of  neuro- 

syphilitics  found  to  show  definite  symptoms  of  syphilis? 
It  is  our  opinion  that  the  situation  in  regard  to  neuro- 
syphilitics  is  the  same  as  for  syphilitics  in  general: 
That  the  same  laws  of  attenuation  of  virus,  and  of 
chance  occur  here  as  elsewhere. 

Just  as  this  book  is  going  to  press,  we  have  learned  that  the 
distraught  Mr.  Jacobs,  still  desirous  of  starting  a  home  for 
himself  and  feeling  entirely  well,  consulted  a  physician. 
This  physician  took  a  sample  of  blood  and  had  it  tested  at  a 
competent  laboratory,  which  reported  the  blood  negative. 

On  the  strength  of  this  test,  the  physician  felt  himself 
warranted  in  recommending,  or  at  least  not  advising  against, 
Mr.  Jacobs'  marriage,  which  has  probably  now  taken  place. 

Although  there  is  some  doubt  what  ethical  relation  a  state 
institution  shall  maintain  with  physicians  in  private  practice, 
we  took  occasion  to  call  the  attention  of  our  patient's  new 
counsellor  to  the  fact  of  the  patient's  neurosyphilis.  We 
noted  that  the  man's  serum  had  been  constantly  positive 


MEDICOLEGAL  AND   SOCIAL  321 

(Massachusetts  State  Board  of  Health  findings)  in  a  score  or 
more  of  observations.  We  called  attention  to  the  fact  that 
lumbar  puncture  had  shown  positive  signs  of  neurosyphilis, 
including  a  positive  W.  R.,  globulin,  excess  albumin,  pleocy- 
tosis,  and  positive  gold  sol.  These  facts,  according  to  a  let- 
ter received  from  the  private  practitioner  in  question,  have 
not  altered  his  opinion  in  the  slightest  to  the  effect  that  our 
patient  is  completely  normal  and  entirely  suitable  for  mar- 
riage. It  is  clear  that  he  regards  the  patient  as  not  a  victim 
of  General  Paresis. 

5.  What  is  the  significance  of  the  negative  observation 
eventually  obtained  in  Jacobs'  serum?  One's  first 
thought  is  to  impugn  the  accuracy  of  the  laboratory 
work,  but  against  this  suspicion  is  the  excellent  repu- 
tation of  the  laboratory  in  question,  and  the  agree- 
ment in  the  majority  of  its  findings  with  those  of  the 
State  Board  of  Health.  It  is  more  likely,  as  we  assured 
the  private  practitioner  at  whose  request  the  observa- 
tion was  made,  that  this  negative  test  was  an  exceptional 
and  isolated  observation  such  as  is  not  infrequent  in 
long  series  of  observations,  particularly  those  made 
under  therapeutic  conditions.  In  so  important  a  mat- 
ter, we  are  inclined  to  feel  that  the  physician  in  question 
should  have  resorted  to  two  more  observations  at  in- 
tervals before  running  counter  to  the  position  taken 
by  the  hospital. 


—  many  a  hard  assay 
Of  dangers,  and  adversities,  and  pains. 


Paradise  Regained,  Book  IV,  lines  47^-479. 


V.  SOME   RESULTS  OF  TREATMENT 

Cases  99-103  show  the  Variety  of  Structural  Lesions  that 
Treatment  has  to  face. 


SPASTIC  HEMIPLEGIA  in  PARETIC  NEURO- 
SYPHILIS  ("general  paresis"),  showing  marked 
degenerative  changes,  a  condition  in  which  therapy 
could  be  theoretically  of  very  little  avail.  Autopsy. 


Case  99.  James  McDevitt  arrived  at  the  Danvers  Hos- 
pital, July  20,  1906  (saying  that  he  came  to  be  "  thawed 
out  "),  and  died  less  than  six  months  later:  January  12,  1907. 
He  was  34  years  of  age.  He  had  been  a  shoe-worker  after 
leaving  school,  had  worked  eight  years  with  the  General 
Electric  Co.,  and  had  then  become  a  bartender.  He  had, 
however,  stopped  work  in  September,  1905,  and  we  may 
safely  say  that  mental  symptoms  had  begun  insidiously  at 
about  that  time.  His  symptoms,  if  there  were  any,  had  been 
masked  by  a  heavy  alcoholism,  but  an  obvious  change  had 
appeared  in  November,  1905.  The  patient  lost  ambition, 
smoked  and  loafed  about  his  room,  and  developed  speech 
disorder.  He  denied  venereal  disease,  nor  was  there  any 
superficial  evidence  of  such. 

Physically,  the  patient  showed  little  or  no  disorder  except 
acne  of  the  trunk,  patches  of  eczema  on  the  left  lower  chest, 
and  numerous  brownish  scars  along  both  tibiae. 

Neurologically,  the  Romberg  position  was  maintained,  but 
the  gait  was  very  unsteady  on  attempts  to  walk  a  straight 
line;  fingers,  tongue,  and  face  were  tremulous,  and  finer 
movements  were  performed  with  marked  incoordination. 
No  direct  or  consensual  light  reactions  could  be  obtained  in 
the  pupils,  which  were  dilated  and  irregular. 

The  condition  of  the  reflexes  is  important  on  account  of 
the  autopsy  findings.  The  abdominal  and  cremasteric  re- 
flexes were  prompt,  and  the  knee-jerks  equal  and  very  lively. 

323 


324 


TREATMENT 


COMMON  THERAPEUTIC  CONCEPTION 

[M]VP  =  TYPICAL  PARESIS 

MV[P]  =  TYPICAL  CEREBROSPINAL  SYPHILIS 

[M]V[P]  =  TYPICAL  SYPHILITIC  ARTERIOSCLEROSIS 


(M  =  Membranes,        V  =  Vessels, 
P  =  Parenchyma,       []  =  not  involved) 


CHART  21 


TREATMENT  325 

Achilles  and  normal  plantar  reactions  were  present;  there 
was  no  clonus ;  the  arm  reflexes  were  very  brisk. 

The  mental  symptoms  need  not  detain  us.  Consciousness 
was  clear;  orientation  for  time,  place,  and  to  some  extent  for 
persons,  was  imperfect.  Arithmetic  had  been  largely  for- 
gotten. Handwriting  was  irregular  and  scrawling,  and  in 
places  unintelligible.  Although  the  patient  claimed  that 
his  memory  was  intact,  it  was  decidedly  imperfect.  He 
remarked  that  John  D.  Rockefeller,  a  Chicago  king,  was  Presi- 
dent; the  General  Electric  Works  had  almost  50,000  people 
at  work ;  and  in  fact  Lynn  was  one  of  the  largest  cities  in  the 
state,  having  over  12,000  people.  The  height  of  patient's 
room  was  estimated  at  25  feet.  There  was  a  slight  eupho- 
ria. There  was  never  any  doubt  of  the  diagnosis  of  PARETIC 
NEUROSYPHILIS  ("general  paresis"). 

Five  months  after  admission,  slight  convulsions  developed, 
after  which  the  patient  was  more  dull  and  demented;  he 
became  bedridden.  More  convulsions  followed,  leaving  the 
right  arm  and  hand  useless.  There  were  clonic  spasms  of  the 
muscles  of  both  lower  legs.  Decubitus  developed  and  death 
occurred. 

We  may  set  the  total  duration  of  symptoms  in  the  case  of 
James  McDevitt  at  a  little  over  a  year;  nor  is  there  any 
evidence  of  previous  or  prodromal  symptoms  beyond  a  total 
period  of  about  15  months,  unless  we  may  regard  his  leaving 
the  General  Electric  Works  to  become  a  bartender  some  nine 
years  before  death,  as  a  symptomatic  change  of  character. 
In  any  event,  it  is  of  note  that  the  autopsy  showed  singularly 
few  lesions.  Death  was  due  doubtless  to  complications 
following  decubitus,  and  there  was  a  slight  acute  splenitis. 
The  kidneys  showed  some  parenchymal  change.  The  aorta 
showed  many  patches  of  sclerosis,  with  calcification  or  ulcera- 
tion  throughout  its  length.  These  changes  were  not  charac- 
teristic of  syphilitic  disease.  There  was  considerable  coronary 
arteriosclerosis  and  a  slight  mitral  valvular  sclerosis.  There 
was  a  brown  atrophy  of  the  heart  muscle,  somewhat  surpris- 
ing in  a  man  of  34  years.  The  brain  was  practically  normal, 
weighed  1200  grams,  and  showed  convolutions  normal  in 
size,  relation,  and  arrangement.  There  was  no  sclerosis 


326  TREATMENT 

grossly  evident  in  the  blood  vessels.  The  pia  mater  appeared 
to  contain  a  considerable  excess  of  clear  fluid.  The  calvarium 
was  of  normal  thickness  and  showed  diploe  and  the  dura 
mater  failed  to  show  adhesions.  There  were  no  macroscopic 
signs  of  lesion  in  the  spinal  cord. 

Microscopically,  the  lymphocytosis,  plasmocytosis,  and 
phagocytosis  of  the  perivascular  spaces,  (relative?)  increase 
in  blood  vessels,  the  gliosis,  and  evidence  of  nerve-cell  destruc- 
tion, taken  together  warranted  the  diagnosis  of  PARETIC 
NEUROSYPHILIS.  It  was  plain  that  the  nerve-cell  destruction 
was  best  marked  in  the  inner  layers  of  the  cortex.  The 
microscopic  study  of  the  spinal  cord  showed  that  there  was 
very  possibly  a  slight  sclerosis  of  the  posterior  columns  in  the 
lumbar  region,  but  this  was  so  slight  that  it  could  hardly  be 
noted  in  the  myelin  sheath  stains  (Weigert).  Very  sharply 
marked,  on  the  other  hand,  were  the  bilateral  pyramidal 
tract  lesions  in  the  lumbar  and  thoracic  regions,  less  marked 
at  the  cervical  levels. 

Without  attempting  to  analyze  carefully  all  these  findings, 
it  is  interesting  to  note  in  this  case  a  foil  to  the  usual  spinal 
cord  picture  of  paretic  neurosyphilis.  The  spinal  cord,  ordi- 
narily normal,  or  perhaps  more  usually  affected  by  a  degree 
of  posterior  column  sclerosis,  in  this  case  showed  such  well- 
marked  pyramidal  tract  sclerosis  that  we  may  perhaps  place 
the  case  in  a  subordinate  group  of  SPASTIC  PARETIC  cases 
of  NEUROSYPHILIS.  The  source  of  the  pyramidal  tract  dis- 
ease lodges,  however,  in  the  cortex  cerebri  itself,  being  part 
and  parcel  of  the  lesions  mentioned  above  as  affecting  more 
directly  the  inner  layers  of  the  cortex.  Many  of  the  so-called 
giant,  or  Betz,  cells  had  undergone  a  complete  destruction. 
It  will  be  remembered  that  clonic  spasms  of  the  muscles  of 
the  legs  appeared  in  the  fortnight  preceding  death,  and  that 
there  had  been  convulsions  for  about  six  weeks  before  death. 
There  was  no  evidence  at  the  autopsy  why  the  right  arm  and 
hand  should  have  become  useless,  whereas  the  left  upper 
extremity  remained  normal.  This  case,  then,  forms  an  ex- 
ception to  the  ordinary  paretic  neurosyphilis  group  in  that 
the  brunt  of  the  microscopic  process  was  borne  by  the  inner 
layers  of  the  cortex.  The  cells  of  origin  of  the  pyramidal 


Bilateral  pyramidal  tract  sclerosis,  secondary  to  destruction  of  large  motor  (Betz) 
cells  of  motor  (precentral)  cerebral  cortex  —  paretic  neurosyphilis. 


TREATMENT  327 

tract  fibres  had  been  cut  in  this  lesion,  and  had  become  sub- 
ject to  partial  or  complete  destruction.  Note,  however, 
that  the  lesion  remained  a  microscopic  one  and  that  the 
marked  convulsions  were  not  related  to  gross  lesions,  thereby 
following  the  rule  for  paretic  seizures. 

From  the  standpoint  of  possible  treatment,  it  is  of  course 
true  that  few  organs  of  the  body  showed  grave  lesions  save  in 
the  calcified  and  ulcerated  aorta,  which  conceivably  might 
have  become  quiescent  under  appropriate  treatment.  But, 
although  the  brain  was  almost  if  not  quite  normal  in  the  gross, 
and  although  its  membranes  showed  practically  no  lesion, 
treatment  would  not  have  been  very  promising.  To  be  sure, 
the  exudate  might  have  been  cleared  away  if  the  spirochetes 
responsible  therefor  had  been  destroyed  by  treatment.  Yet 
the  destruction  of  the  giant  cells  of  origin  of  the  pyramidal 
tract  fibres  to  such  an  extent  as  in  this  case  could  hardly 
have  been  compensated  for  by  any  known  process.  So  far 
as  we  are  aware,  the  destruction  of  considerable  numbers  of 
the  smaller  association  elements  of  the  brain  is  subject  to  the 
compensation  of  other  elements  of  the  nervous  system,  which 
conceivably  might  be  re-educated  or  newly  educated  to  per- 
form certain  processes.  The  histological  picture  in  a  case 
like  that  of  Me  Devi  tt  accordingly  leads  to  the  hypothesis 
that  so  well  marked  a  spastic  paresis,  even  in  the  presence 
of  otherwise  favorable  signs,  would  be  of  especially  baneful 
portent  therapeutically. 


328  TREATMENT 


NEUROSYPHILIS  with  total  duration  of  symp- 
toms twenty-two  days.  The  comparatively  MILD 
BRAIN  LESIONS,  INFLAMMATORY  AND  NOT  DE- 
GENERATIVE in  type,  suggest  the  possibility  that 
therapy  might  have  been  successful.  Autopsy. 


Case  100.  Jacob  Methuen,  35,  was  a  carpenter  from 
Newfoundland.  He  was  working  upon  a  certain  Thursday 
with  his  brother,  who  noticed  that  Jacob  was  lifting  the  tools 
about  in  an  unusual  manner  and  talking  strangely  to  his 
fellow  workmen.  He  fell  asleep,  going  home  in  the  street  car, 
and  said  afterward  that  he  felt  dazed  and  peculiar.  He  talked 
all  kinds  of  nonsense  to  his  wife  upon  arrival.  Methuen 
remained  in  bed  next  day,  fancying  he  was  going  to  die, 
calling  his  family  together,  and  saying  good-bye  to  them. 
He  remained  in  bed  all  through  the  next  day,  but  on  Sunday 
appeared  better, — more  active,  and  in  fact  quite  natural. 
He  slept  only  an  hour  Sunday  night,  calling  to  his  wife  that  it 
was  time  to  get  up.  On  Monday  he  began  to  be  irritable 
to  his  wife,  and  accused  her  of  flirting  with  his  brother  and 
intending  to  elope  with  him.  He  struck  his  wife  several 
times,  and  when  two  brothers  came  to  watch  him,  accused 
them  both  of  trying  to  steal  his  wife,  and  struck  them.  Tues- 
day he  remained  in  bed  until  late  at  night,  when  he  arose 
and  tried  to  assault  the  family. 

It  seems  that  another  brother  of  the  patient  had  died  but 
eleven  days  before  his  admission  to  the  hospital  and  five  days 
before  the  onset  of  Jacob's  symptoms.  Since  his  brother's 
death  he  had  been  dwelling  upon  religious  matters,  and  in 
fact  the  day  after  his  brother's  death,  he  waked  up  during 
the  night,  saying  that  he  was  too  happy  to  sleep,  that  he 
heard  the  Master's  voice,  and  at  times  the  devil's  voice; 
that  there  was  to  be  a  modern  miracle  and  his  spiritual  life 
from  now  on  would  be  different. 

Eleven  days  after  admission  to  the  hospital,  Methuen 
died,  making  a  total  duration  of  symptoms,  beginning  at  his 
brother's  death,  of  22  days. 


TREATMENT  329 


NETTROSYPHILITIC  LESIONS 

LESIONS  OF  THE  SECONDARY  PERIOD 

(1)  INTERSTITIAL  ENCEPHALITIS  OR  MYELITIS 

("meningitis") 

(2)  PARENCHYMATOUS  ENCEPHALITIS  OR  MYELITIS 

("encephalitis,"  "myelitis") 

LESIONS  OF  THE  TERTIARY  PERIOD 

(1)  CHRONIC  INTERSTITIAL  ENCEPHALITIS  OR  MYELITIS 

("gummatous  meningitis  ") 

(2)  CHRONIC  PARENCHYMATOUS  ENCEPHALITIS 

("  dementia  paralytica  ") 

(3)  CHRONIC  PARENCHYMATOUS  MYELITIS 

("  tabes  dorsalis  ") 


"We  have  shown  that  the  central  nervous  system  is  affected  by  syphilis 
at  the  same  periods  and  in  the  same  manner  as  are  other  internal  organs. 
In  addition  the  '  parasyphilitic '  lesions  are  also  of  a  typically  syphi- 
litic nature,  being  directly  comparable  to  the  parenchymatous  affections 
found  elsewhere  in  the  body.  They  are  'tertiary'  lesions  differing 
only  from  the  so-called  'gummatous'  processes  in  the  central  nervous 
system  in  that  their  localization  is  in  the  parenchyma  while  that  of  the 
latter  is  in  the  interstitial  tissues. " 

MclNTOSH  AND  FlLDES,  1914 


CHART  22 


33O  TREATMENT 

Physical  examination  showed  a  man  5'  9"  tall,  weighing 
149  pounds,  rather  pale  and  poorly  nourished,  with  a  some- 
what enlarged  heart  and  no  evidence  of  venereal  disease. 

Neurologically  there  was  a  slight  facial  and  digital  tremor, 
but  otherwise  no  symptom  or  reflex  disorder  except  that  the 
tendon  reflexes  were  generally  increased;  the  knee-jerks 
especially  were  very  vigorous.  There  was  no  speech  defect. 
His  handwriting  was  fairly  legible. 

The  patient  was  very  noisy  and  uncontrollable,  tearing 
clothing  and  biting,  striking  the  attendants,  refusing  food, 
talking  rapidly,  loudly,  and  incoherently.  His  manner  sug- 
gested auditory  hallucinations  but  no  positive  evidence  of 
these  was  obtained.  His  clothes  could  not  be  kept  on  him. 
The  following  is  a  sample  of  his  reactions:  As  the  examiner 
entered,  the  patient  stood  stark  naked  and  glaring.  He 
started  to  talk  as  follows:  "  Methuen,  —  I,  Saviour,  come 

to  life  and  ought  to  die Now  I  lay  me  -  -  Now  I 

die The  heart  beats No,  I  ain't  going  to  die 

I  am  going  out  soon.  I  want  my  clothes  -  -  You 

can't  hold  me;  I  am  strong."  (Struggles  violently  with  the 
attendants.)  "  I  am  God.  God.  I  know  you,  you  can't 

fool  me. I  am  here I  can  do  you  all.  How 

many  doctors  are  there  here?  "  (Struggles  violently.  Looks 
at  examiner.)  "  He  is  writing  something.  Sir,  you  can't  fool 
me  in  a  million  years.  Do  you  understand  that,  doctor? 
You  can't  fool  me.  Write  all  the  prescriptions  you  want  to. 
Ten  thousand  years;  you  hear  that,  doctor?  Ten  thousand 
years.  You  can't  fool  me;  ten  thousand  years.  Ten  thou- 
sand years  are  but  a  day  for  the  spirit  of  the  Lord/'  etc.,  etc. 

The  excitement  continued  unabated.  The  patient  became 
entirely  disoriented,  and  finally  almost  unable  to  move. 
He  lay  in  bed  trying  to  talk  and  muttering  broken  gibberish, 
still  attempting  to  struggle  to  the  extent  of  his  limited 
strength. 

The  autopsy  showed  no  sign  of  lesion  (brain  weight  1380 
grams),  unless,  perhaps,  the  occipital  regions  were  slightly 
firmer  than  the  rest  of  the  brain.  Death  was  apparently 
due  to  a  bilateral  pneumonia,  bronchial  type.  There  was 
an  acute  splenitis.  The  only  chronic  lesions  of  the  body 


Paretic  neurosyphilis  (  "general  paresis"  )  macroscopically  normal,  mi- 
croscopically characteristic.  Treatment  does  not  have  to  face  massive 
destructive  processes  already  complete. 


TREATMENT  33! 

were  a  bilateral  chronic  adhesive  pleuritis  and  a  slight  scler- 
osis of  the  arch  of  the  aorta. 

Microscopically  there  was  a  distinct  though  mild  degree 
of  lymphocytosis  of  the  perivascular  spaces  in  many  regions. 
Somewhat  extended  search  failed  to  reveal  plasma  cells,  and 
it  is  certain  that  if  plasma  cells  existed,  they  must  have 
occurred  in  very  small  numbers. 

Here,  then,  was  a  case  of  DIFFUSE  NEUROSYPHILIS  (with 
brain  picture  consistent)  with  symptoms  lasting  but  22  days 
and  with  an  appearance  of  acute  mania.  It  is  to  be  noted 
that  this  case  arrived  at  the  hospital  on  the  eleventh  day  of 
his  symptoms.  The  case  occurred  long  before  the  develop- 
ment of  the  temporary  care  system  in  Massachusetts.  It  is 
probable,  or  at  any  rate  possible,  that  he  would  have  been 
brought  to  the  hospital  far  earlier,  say,  upon  the  sixth  day, 
had  the  modern  temporary  care  system  been  installed  at 
that  time.  The  routine  W.  R.  examination  would  then 
have  been  made.  With  more  effective  hydrotherapy,  it  is 
possible  that  the  patient's  life  might  have  been  prolonged 
and  that  treatment  might  have  been  effective.  So  far  as 
we  can  see,  the  case  would  have  been  a  singularly  good  one 
for  treatment  despite  the  practical  unmanageability  of  the 
case  under  ordinary  home  treatment,  and  even  under  hos- 
pital conditions  where  modern  hydrotherapeutic  appliances 
are  not  available. 


332 


TREATMENT 


PARETIC  NEUROSYPHILIS  showing  very 
MARKED  MENINGITIS,  suggesting  that  therapy 
might  have  produced  improvement.  Autopsy. 


Case  101.  We  report  the  case  of  John  Baxter,  a  boat 
tender  of  48  years,  because  this  particular  victim  of  PARETIC 
NEUROSYPHILIS  seems  to  have  had  the  most  markedly  thick- 
ened and  altered  meninges  in  our  whole  series.  Of  course,  the 
therapeutic  theory  upon  which  we  now  proceed  in  the  treat- 
ment of  non-paretic  and  possibly  even  of  paretic  neuro- 
syphilis  is  that,  other  things  being  equal,  the  meningitis 
can  be  removed  by  treatment,  or  in  the  course  of  treatment, 
so  that  the  degree  of  ultimate  recovery  rather  depends  upon 
the  condition  of  the  brain  substance  itself  than  upon  the 
condition  of  the  meninges.  Here,  at  all  events,  is  an  example 
of  the  most  highly  meningitic  neurosyphilis  that  we  have 
seen. 

Curiously  enough,  two  of  Baxter's  brothers  were  also 
patients  at  the  hospital  at  which  Baxter  died,  and  a  number 
of  the  other  members  of  the  family  are  reported  as  "nervous." 
It  seems  that  at  35  Baxter  began  to  drink  heavily  and  had 
never  given  over  the  habit  of  alcoholism. 

Upon  admission  to  the  hospital,  in  fact,  he  showed  a  suf- 
ficiently typical  picture  of  delirium  tremens.  His  conscious- 
ness was  clouded,  he  had  vivid  visual  hallucinations  and  was 
very  apprehensive. 

His  heart  was  enlarged  to  the  left;  the  pulse,  120,  was  of 
increased  tension  and  irregular;  there  was  peripheral  arterio- 
sclerosis; the  teeth  were  poor;  the  tongue  coated;  and 
the  mouth  foul.  The  urine  showed  a  trace  of  albumin  and 
rare  hyalin  casts. 

Neurologically,  the  gait  was  somewhat  unsteady,  there 
was  an  extreme  tremor  of  the  whole  body,  including  the 
tongue  and  fingers.  The  Romberg  sign  was  negative  although 
there  was  marked  swaying.  The  pupils  were  equal  and  reacted 
normally;  the  knee-jerks  were  markedly  exaggerated,  the 


A  high  degree  of  chronic  leptomeningitis.     Pia  mater  thick,  opaque, 
concealing  brain.     In  paretic  neurosyphilis  ("general  paresis"). 


TREATMENT  333 

arm  reflexes  somewhat  exaggerated.    The  remainder  of  the 
reflexes  upon  systematic  examination  were  negative. 

Upon  arrival,  Baxter  was  put  to  bed,  but  he  barricaded 
his  door  and  fought  with  the  attendants.  The  tremor  in- 
creased, the  hallucinations  were  both  visual  and  auditory. 
After  a  few  days,  Baxter  became  so  weak  that  he  could  not 
move.  He  refused  to  eat  for  a  period  of  two  days,  explain- 
ing in  whispers  that  he  did  not  wish  to  be  poisoned;  a  voice 
had  told  him  the  food  was  to  be  poisoned.  The  voice  was  of 
agreeable  tones,  probably  belonging  to  a  lady;  it  did  not 
speak,  but  sang  to  him.  The  clouding  of  consciousness 
failed  to  clear  up,  as  in  delirium  tremens,  so  that,  though 
patient  was  admitted  March  3d,  it  was  hardly  possible  to 
speak  freely  with  him  until  more  than  a  month  later,  April  9th. 
A  goodnatured  conversation  would  run  as  follows- 

"What  is  your  name?"       "  Baxter." 

" First  name? "  After  long  pause,  "Don't  know." 

"John?"  Pause  of  7  seconds,  "Yes,  I 

think  it  is." 

"How  old  are  you?"  "  There  are  legs there  is  a 

body up  to  here " 

"Say  the  alphabet."  Term  not  understood. 

"Say  the  a,  b,  c."  "  Oh  yes;  a,  b,  c,  d  (long 

pause),  e,  f ;  I  cannot  say  it, 
I  did  not  have  much  educa- 
tion; I  am  not  intelligent." 
(In  point  of  fact,  the  patient 
had  a  good  grammar-school 
education,  and  had  long 
worked  as  a  clerk  in  a  gro- 
cery store,  with  good  wages.) 
There  was  some  speech  defect. 

Soon  the  hallucinatory  phase  passed,  and  the  patient 
remained  in  a  cloudy  and  disoriented  state,  inaccessible, 
rarely  speaking,  and  gradually  failing  physically.  Death 
occurred  about  three  months  after  admission  (pulmonary 
symptoms). 

In  estimating  the  duration  of  the  process  in  John  Baxter, 
we  must  take  into  account  that  he  left  the  grocery  business 
and  became  a  hard-working  but  poorly-paid  boat  tender  at 


334  TREATMENT 

about  35  years,  at  the  same  time  that  the  alcoholic  habit 
began. 

The  autopsy  showed  that  death  was  due  to  bronchopneu- 
monia  with  pleurisy.  There  were  in  the  body  a  variety 
of  chronic  lesions,  such  as  gastritis,  colitis,  epididymitis, 
splenitis,  parietal  and  valvular  endocarditis,  prostatitis,  chronic 
appendicitis,  and  some  mesenteric  lymphnoditis.  The  heart 
was  somewhat  hypertrophied.  There  was  a  slight  diffuse 
nephritis  with  cysts,  emaciation,  and  decubitus.  The  cal- 
varium  was  thick  and  somewhat  dense.  The  dura  was 
thickened  and  adherent,  and  the  pia  mater,  —  as  above 
stated,  the  most  thickened  and  altered  pia  mater  in  our 
series,  —  is  described  as  everywhere  thickened,  of  a  brownish 
gray  and  white  color,  especially  over  the  vascular  lines,  and 
as  showing  small  white  areas  of  deeper  thickening  scattered 
over  the  surface,  but  most  markedly  over  the  sulci,  and  not 
as  a  rule  over  the  crowns  of  the  gyri.  There  were  also  yellow- 
ish brown  spots  with  a  suggestion  of  fibrin  over  the  lateral 
aspects  of  both  hemispheres.  The  vessels  at  the  base  were 
not  remarkable  in  the  gross.  The  brain  weighed  1220  grams, 
and  appeared  to  be  of  darker  color  than  usual. 


TREATMENT 


335 


Some  cases  of  PARETIC  NEUROSYPHILIS 
("general  paresis")  have  so  much  BRAIN 
ATROPHY  that  it  is  not  possible  to  expect  much 
improvement  through  antisyphilitic  therapy. 


Case  102.  Theodosia  Jewett,  dead  at  58  years,  showed  the 
most  remarkably  wasted  brain  in  a  long  series  of  victims  of 
paretic  neurosyphilis.  We  present  her  case  to  emphasize 
what  therapy  must  face  in  certain  instances,  but  would 
recall  the  fact  that  exceedingly  few  such  wasted  brains  have 
come  to  our  attention  in  cases  dying  in  the  institutions  of 
Massachusetts. 

Mrs.  Jewett,  a  housewife,  whose  parents  died  of  shock,  and 
one  of  whose  two  brothers  also  died  of  shock,  was  a  normal 
child  and  schoolgirl,  and  worked  as  dressmaker  until  she 
was  married,  at  24,  to  a  grocer,  by  whom  she  had  two  children. 
At  the  age  of  46,  Mrs.  Jewett  began  to  suffer  from  so-called 
"  nervous  prostration."  The  attack  lasted  some  two  years, 
but  there  were  no  psychotic  symptoms  beyond  worry  and 
insomnia.  The  menopause  occurred  at  52,  at  which  time 
the  first  signs  of  psychosis  appeared,  namely,  a  forgetfulness 
concerning  familiar  matters,  such  as  sewing,  cooking,  and 
the  like.  At  55,  this  amnesia  had  become  so  marked  that 
Mrs.  Jewett  could  neither  write  nor  tell  time.  She,  however, 
was  a  perfectly  quiet  and  easily  manageable  patient,  often 
subject  to  drowziness  in  the  day. 

Six  months  before  her  admission  to  the  hospital,  she  began 
to  suffer  from  insomnia,  failed  to  recognize  her  surroundings, 
and  had  a  number  of  crying  spells.  Restlessness  had  begun 
a  month  before  admission ;  auditory  hallucinations  developed 
in  the  form  of  imaginary  conversations  with  dead  persons. 
A  certain  loquacity  set  in,  and  for  a  week  before  admission, 
Mrs.  Jewett  became  somewhat  resistive. 

Physically,  the  patient  was  sallow,  poorly  nourished,  with 
pale  mucous  membranes,  peripheral  arteriosclerosis,  no  teeth, 
muscular  feebleness,  tremor  of  hands  and  tongue,  and  active 


336  TREATMENT 

knee-jerks.  Mentally,  the  patient  was  depressed,  talked  to 
herself,  assumed  a  supplicating  position,  suddenly  altered 
her  attitude,  and  was  very  tremulous.  Her  talk  was  low, 
mumbling,  and  incoherent,  for  the  most  part  composed  of 
answers  to  her  own  questions.  Sometimes  there  was  a 
curious  difficulty  in  speaking,  such  that  the  lips  moved  but  no 
sound  emerged ;  but  for  the  most  part  there  was  no  difficulty 
in  uttering  words.  The  patient  either  could  or  would  not 
write.  Only  when  the  attention  was  secured  by  speaking  to 
her  sharply  was  she  apparently  able  to  understand  questions, 
and  the  answers  to  these  sharp  questions  came  spasmodically 
and  as  if  interrrupting  her  own  thoughts.  Nor  was  it  ever 
possible  to  obtain  a  repetition  of  the  same  answer. 

The  patient  died  in  exhaustion,  with  pulmonary  symptoms 
three  weeks  after  admission. 

The  autopsy  which  was  performed  3!  hours  after  death 
showed  the  following  points  of  interest: 

The  heart  weighed  210  grams.  There  was  marked  thick- 
ening of  the  aortic  valve.  The  coronaries  were  slightly 
thickened. 

The  lungs  were  slightly  adherent  to  the  chest  wall  at  the 
apices  and  posteriorly.  The  right  lung  was  consolidated 
in  the  lower  two  lobes  posteriorly  and  the  bronchi  exuded 
pus ;  the  left  lung  was  not  remarkable.  There  was  a  chronic 
splenitis. 

The  liver  showed  fibrous  changes,  was  a  brownish-red  in 
color,  mottled  with  yellow. 

Combined  weight  of  the  kidneys  195  grams.  The  capsules 
were  adherent,  tearing  the  cortex  when  stripped. 

The  diploe  were  well  marked.  The  dura  was  not  adherent. 
The  pia  was  slightly  thickened  and  raised  from  the  cortex  by 
a  large  amount  of  subpial  fluid  (showing  atrophy  of  the  cortex). 
The  pial  vessels  were  injected,  more  markedly  so  on  the  left 
side.  The  arachnoid  villi  were  reported  as  moderately  de- 
veloped, especially  along  the  longitudinal  fissure. 

The  brain  was  rather  soft  in  all  regions.  The  weight 
was  1045  grams.  According  to  Tigges'  formula  the  weight 
of  the  brain  should  be  approximately  8  times  the  body  length 
in  centimeters.  The  length  in  this  case  was  1 58  cm.,  therefore, 


;".  <  "s,  •"*'*- 


..-M 


-.  ••  .  , 


Perivascular  exudate  (low  power) 
in  atrophic  cortex  from  case  of  gen- 
eral paresis. 


Markedly  atrophic  cortex,  but  with- 
out local  perivascular  exudate. 


TREATMENT  337 

according  to  this  formula  the  weight  of  the  brain  should  have 
been  1464  grams.  The  difference  of  more  than  400  grams 
is  evidently  a  loss  to  be  accounted  for  by  atrophy,  a  very 
heavy  loss. 


1.  Was   the   "  nervous   prostration "   at   46   of   syphilitic 

origin?  One  cannot  give  a  categorical  answer  to  this 
question.  The  high  incidence  of  shock  in  the  family 
suggests  poor  stock  in  which  a  psychoneurosis  is  not 
an  unusual  phenomenon.  The  presence  of  syphilis 
might  act  as  a  debilitating  factor  or  agent  provocateur, 
if  it  were  not  to  cause  any  demonstrable  brain  lesion. 
As  pointed  out  in  the  case  of  Harrison  (9),  however, 
it  is  not  unusual  in  neurosyphilis  to  find  a  history  of 
symptoms  occurring  years  before  the  final  breakdown 
and  symptoms  frequently  not  recognized  as  of  neuro- 
syphilitic  nature. 

2.  Does  the  fairly  long  duration  of  the  psychosis  (at  least 

3  years)  explain  the  marked  atrophy?  Cases  having 
symptoms  even  much  longer  than  three  years  at  times 
show  relatively  very  little  atrophy,  so  that  this  factor 
in  itself  cannot  be  said  to  explain  the  tremendous 
destruction  of  tissue. 


338  TREATMENT 


The  THERAPY  OF  NEUROSYPHILIS  has  to  face 
not  merely  variations  in  the  degree  of  brain 
wasting  and  in  the  degree  of  meningitis,  but  also 
variations  in  the  topographical  distribution  of 
lesions.  Autopsy. 


Case  103.  To  bring  out  this  point  we  may  instance  the 
case  of  Alfred  Weed,  a  victim  of  PARETIC  NEUROSYPHILIS, 
dying  at  the  age  of  48  years  after  a  course  of  about  seven 
years.  The  following  is  an  abstract  of  the  clinical  history: 

A.  W.  suffered  from  lues  some  24  years  before  his  death  at 
Danvers  Insane  Hospital  in  1907.  There  is  no  account  of 
insanity  in  his  family.  The  patient  had  been  undergoing 
mental  changes  for  six  years  before  death.  At  the  age  of  42 
began  to  take  interest  in  socialism  and  spiritualism.  Would 
become  excited  at  times  and  was  observed  to  talk  to  himself. 
At  times  it  seemed  that  he  was  reacting  to  visual  hallucina- 
tions. After  eight  months  he  became  depressed  and  appre- 
hensive and  developed  delusions  of  poisoning. 

On  admission  to  the  Danvers  Insane  Hospital  in  June, 
1902,  the  subject  was  found  to  be  ataxic,  falling  in  the  Rom- 
berg  position.  Pupils  were  equal  but  of  pin-point  size. 
There  was  tremor  of  the  facial  muscles.  The  knee-jerks  were 
absent.  Speech  was  ataxic.  Memory  defective.  Depressed. 
Thought  he  was  to  be  punished.  Refused  to  eat. 

Later  in  the  year  of  admission,  patient  became  more 
negativistic.  He  refused  to  have  his  clothes  brushed.  His 
answers  were  slow.  Mental  arithmetic  was  correctly  but 
slowly  done.  During  January,  1903,  the  patient  was  apt  to 
be  active  and  talkative  for  a  time,  and  then  his  attitude 
would  suddenly  change  to  one  of  silence,  resistivement  and 
untidiness.  From  time  to  time  he  would  be  querulous 
and  sulky.  In  August,  1903,  the  patient  became  weaker 
and  could  walk  with  assistance  only.  Paralysis  developed 
in  the  left  facialis  region  and  in  the  left  external  rectus. 
Pupils  were  still  small,  but  the  left  had  become  smaller  than 


TREATMENT  339 

the  right.  Light  reaction  tests  unsatisfactory.  Knee-jerks 
could  not  be  obtained. 

In  December,  1903,  the  patient  was  untidy  and  helpless, 
lying  with  his  thighs  and  legs  flexed.  The  limbs  were  spastic 
on  passive  motion.  In  1905,  the  pain  sense  of  the  legs  was 
found  lost  and  the  pupils  were  small  and  stiff.  The  pro- 
truded tongue  was  deflected  to  the  right.  The  right  labial 
fold  was  more  prominent  than  the  left.  Knee-jerks  remained 
absent.  Ataxia  was  extreme. 

The  Neurological  Findings  may  be  summed  up  as  follows: 

1 .  Ataxia  of  the  legs. 

2.  (Probable)  Diminished  sensibility  in  the  legs. 

3.  Pupils  small  and  stiff.     Left  smaller  than 

the  right. 

4.  Paralysis  of  left  facialis. 

5.  Paralysis  of  left  external  rectus. 

6.  Tongue  protruded  to  right. 

7.  Right  elbow  jerk  greater  than  left. 

8.  Knee-jerks  absent. 

The  cause  of  death  was  bronchopneumonia.  The  walls 
and  valves  of  the  heart  showed  a  few  chronic  changes.  There 
was  a  marked  splenitis  and  an  atrophy  of  the  liver.  The 
kidneys  showed  numerous  depressed  scars.  The  arch  of  the 
aorta  was  somewhat  sclerotic.  The  following  is  a  full  de- 
scription of  the  head  findings  which  we  present  by  way  of 
comparison  with  other  cases.  Note  especially  the  cerebellar, 
dentate,  and  olivary  changes.  Note  also  the  fact  that  pal- 
pable sclerosis  is  demonstrable  over  a  far  larger  area  than 
atrophy,  so  that  we  may  almost  safely  conclude  that  the  proc- 
ess of  induration  sometimes  precedes  that  of  atrophy.  One 
gets  the  impression  from  the  extent  of  visible  atrophy  and 
tangible  induration  in  this  case,  that  a  possible  therapy  would 
have  not  merely  to  clear  the  perivascular  spaces  of  cells  and 
spirochetes,  but  would  also  need  to  arrest  the  indurating  and 
wasting  process.  Nor  could  any  therapy  deal  effectively  with 
the  superior  frontal  and  upper  central  atrophy  of  the  cerebrum 
of  this  case,  or  with  the  olivary  and  cerebellar  lesions. 

Head:     Hair  thin  at  vertex.     Scalp  normal.     Calvarium 


34O  TREATMENT 

thin  and  dense.  Dura  mater  slightly  adherent  to  calvarium 
at  vertex.  Sinuses  normal.  Arachnoidal  villi  well  devel- 
oped. Pia  mater  of  anterior  and  central  regions  contains  an 
excess  of  fluid.  The  pial  veins  well  injected. 

The  pia  mater  exhibits  one  unusual  lesion :  Faintly  yellow- 
ish brown  spots  of  miliary  and  slightly  larger  size  are  scat- 
tered irregularly  in  clusters  over  the  vertex.  These  miliary 
pial  macules  are  observed  especially  over  the  posterior  third 
of  the  left  superior  frontal  gyrus  (a  group  of  twelve  or  more). 
Two  are  seen  in  the  pia  mater  of  the  right  superior  frontal 
gyrus.  One  is  seen  in  the  upper  part  of  the  left  post  central 
gyrus.  The  upper  end  of  the  right  postcentral  gyrus  con- 
tains three  macules. 

Besides  these  brownish  macules,  the  pia  mater  also  shows 
focal  white  thickenings  which  resemble  the  more  frequent 
appearances  of  chronic  fibrous  leptomeningitis.  The  white 
thickenings  are  of  irregular  size  but  are,  as  a  rule,  larger  than 
the  macules  above  mentioned.  They  occur,  as  a  rule,  over 
the  sulcal  veins  and  are  most  frequent  in  the  anterior  region. 

The  vessels  at  the  base  are  normal.  There  is  no  evidence 
of  pial  thickening  at  the  base  of  the  brain.  Brain  weight, 
1265  grams.  There  is  visible  atrophy  of  both  superior  frontal 
gyri  and  of  the  upper  two-thirds  of  both  central  gyri.  The 
extent  of  palpable  sclerosis  surpasses  that  of  visible  atrophy. 
Palpable  increase  of  consistence  is  shown  by  the  prefrontal, 
orbital  (more  marked  on  left  side),  frontal,  central,  hippo- 
campal  and  occipital  regions.  The  temporal  cortex  is  of 
normal  or  slightly  reduced  consistence. 

Section  of  the  cerebral  cortex  shows  everywhere  preserva- 
tion of  the  cortical  markings.  The  sclerosed  areas  show  a 
diminution  in  depth  of  the  cortex,  which  is  more  marked  in 
the  left  prefrontal  region.  The  white  matter  of  the  centrum 
semiovale  of  the  prefrontal  and  occipital  regions  on  both 
sides  shows  an  increase  of  consistence.  The  cerebellar  cortex 
also  shows  variations  in  consistence.  The  clivus  and  lobus 
cacuminis  and  the  posterior  half  of  the  inferior  surfaces  of 
both  cerebellar  hemispheres  are  firmer  than  normal.  The 
laminae  of  the  left  clivus  are  a  trifle  narrower  than  those  of 
the  right.  There  is  visible  extensive  atrophy  of  the  laminae 


TREATMENT  341 

on  both  sides  of  a  fissure  in  the  middle  of  the  left  lobus  cacu- 
minis.  In  the  coordinate  portion  of  the  right  cacumen  there 
is  a  similar  process  which  is  less  marked.  The  dentate  nuclei 
are  firm.  The  olives  show  an  increase  of  consistence,  equal 
on  both  sides.  The  left  olive  shows  on  section  a  crowding 
together  of  its  folds  in  the  middle  part  of  the  upper  limb. 
Spinal  cord  was  not  remarkable. 

Summary: 

Adhesive  pachymeningitis 

Chronic  fibrous  leptomeningitis 

Miliary  pial  macules 

Cerebral  atrophy 

Cerebral  sclerosis 

Cerebellar  atrophy  and  sclerosis 

Bronchopneumonia 

Chronic  splenitis 

Nephritis 

Aortitis 


342  TREATMENT 


It  is  generally  recognized  that  DIFFUSE  NEURO- 
SYPHILIS  ("  cerebrospinal  syphilis  ")  frequently 
is  cured  through  antisyphilitic  therapy.  Example. 
Mental  improvement,  hi  one  month;  recovery  from 
paralysis,  ten  months. 


Case  104.  John  Edwards,  a  man  of  28  years,  well  de- 
developed  and  nourished,  with  general  enlargement  of  glands 
and  skin  lesions,  came  to  the  hospital  in  a  stuporous  con- 
dition, with  evidences  of  a  complete  hemiplegia. 

According  to  the  wife,  Edwards  had  had  a  chancre  of 
the  lip  about  a  year  before,  for  which  he  had  been  treated 
with  an  intravenous  injection,  presumably  of  salvarsan,  and 
also  presumably  with  mercury.  The  lip  lesion  had  then 
disappeared.  For  a  month  before  admission,  Edwards  had 
had  headache  and  dizziness,  for  which  he  was  given  pills 
and  drugs.  There  had  also  been  difficulty  with  speech  and 
numbness  of  the  left  arm  as  far  up  as  the  elbow,  but  this 
paresthesia  had  quickly  disappeared.  The  hemiplegia  was 
of  only  a  few  days'  duration.  After  a  feeling  of  nausea  and 
vomiting,  the  patient  had  fallen  with  left-sided  paralysis. 
Afterwards,  he  had  shown  mental  peculiarities,  eventually 
becoming  noisy,  hard  to  manage,  and  appropriate  for  hos- 
pital care. 

The  physical  examination  showed  a  variety  of  increased 
reflexes,  including  ankle-clonus  on  the  left  side. 

The  question  might  arise  whether  this  case  was  one  of 
hemorrhage  or  thrombosis,  and  the  facts  about  the  onset 
of  the  hemiplegia  are  inadequate  for  a  decision.  However, 
at  so  early  an  age,  the  probability  of  syphilis  is  large  and  the 
history  of  labial  chancre  was  quite  suggestive.  If  we  may 
conclude  neurosyphilis,  the  diagnosis  of  thrombosis  rather 
than  rupture  of  blood  vessel  is  likely.  The  laboratory  tests 
bore  out  the  diagnosis  since  the  W.  R.  of  serum  and  fluid 
both  proved  positive;  the  gold  sol  reaction  was  syphilitic; 


TREATMENT  343 


NON-PARETIC   NEUROSYPHILIS 

DIFFUSE  NEUROSYPHILIS,  MENINGOVASCULAR  PAREN- 
CHYMATOUS,  CEREBROSPINAL  SYPHILIS 

CASES  SYSTEMATICALLY  TREATED  13 

CLINICAL  RECOVERY,  C.S.F.  NEGATIVE  II 

UNIMPROVED  I 

UNIMPROVED,   BUT  C.S.F.   NEGATIVE  I 


MASSACHUSETTS  COMMISSION  ON  MENTAL  DISEASES, 

November,  1916 


CHART  23 


344  TREATMENT 

there  were  176  cells  per  cmm.;  there  was  excess  albumin, 
and  a  positive  globulin  reaction. 

The  outcome  in  such  a  case  is  dubious.  If  death  does 
not  occur  soon,  recovery  is  not  impossible  under  treatment. 
At  all  events,  a  considerable  improvement  is  likely. 

Edwards  was  given  bi-weekly  injections  of  salvarsan, 
intramuscular  injections  of  mercury  salicylate,  and  doses  of 
potassium  iodid,  averaging  100  grains,  three  times  a  day. 
Under  this  treatment,  he  slowly  recovered  and  became 
mentally  clear  after  a  few  weeks.  The  paralysis  seemed 
complete  and  permanent.  Even  after  three  or  four  months, 
there  was  absolutely  no  change  in  the  condition,  and  Edwards 
was  quite  unable  to  move  either  arm  or  leg.  Meanwhile, 
the  spinal  fluid  had  become  practically  negative  to  all  tests. 

Treatment  was  somewhat  optimistically  continued  and 
was  rewarded  at  the  end  of  ten  months  with  marked  im- 
provement such  that  the  patient  was  able  to  stand  on  the 
paralyzed  leg  and  move  the  arm  to  a  certain  degree.  This 
improvement  is  still  continuing.  The  spinal  fluid  and  the 
serum  have  remained  negative  to  laboratory  tests. 

Note:  A  period  of  six  months  is  commonly  regarded  as 
that  period  in  which  improvement  in  paralysis  is  to  occur 
if  there  is  to  be  any  improvement.  There  was  certainly 
not  the  slightest  improvement  in  the  paralysis  of  this  case 
before  eight  or  nine  months  of  treatment  had  elapsed,  and 
it  took  ten  months  to  secure  the  marked  improvement 
mentioned. 

1.  What  is  the  significance  of  the  prodromal  symptoms? 

The  headache  and  dizziness  should  have  been  viewed 
with  great  gravity.  They  are  characteristic  in  MEN- 

INGOVASCULAR   NEUROSYPHILIS. 

Moreover  in  this  case  there  had  also  been  difficulties 
with  speech  and  other  transient  symptoms  which 
should  have  called  attention  far  earlier  to  the  possi- 
bility of  neurosyphilis. 

2.  What  is  the  significance  of  the  high  cell  count:    176  per 

cubic  millimeter?  Such  high  cell  counts  are  frequent 
enough  in  diffuse  neurosyphilis,  but  low  cell  counts  are 
frequent  also.  But  although  the  high  cell  count  taken 
alone  is  of  lesser  significance,  the  fact  that  the  high 


TREATMENT  345 

cell  count  in  this  case  is  associated  with  a  "  syphilitic  " 
gold  sol  reaction  is  of  far  greater  significance  for  diag- 
nosis. These  associated  findings  are  characteristic  of 
meningovascular  neurosyphilis. 

3.  What  kind  of  recovery  may  be  expected  in  successful 

examples  of  treatment  in  meningovascular  cases? 
Recovery  with  defect.  It  will  be  noted  that  ten 
months  elapsed  before  any  marked  improvement  oc- 
curred on  the  paralyzed  side.  We  could  not  expect  a 
complete  recovery  from  this  paralysis. 

4.  Was   inadequacy   of   treatment   following   the   chancre 

responsible  for  the  early  cerebrospinal  involvement? 
In  this  connection  one  must  remember  that  such  neural 
involvements  occur  occasionally  even  during  active 
treatment  (neurorecidives) .  The  discontinuance  of 
treatment  after  a  short  period,  in  this  case  less  than 
a  year,  is  always  a  risk  to  say  the  least.  And  this  is 
true  even  though  the  W.  R.  becomes  negative,  for 
trouble  of  a  neurosyphilitic  nature  may  occur  later; 
this  when  both  blood  and  spinal  fluid  have  previously 
been  found  negative.  The  old  rule  of  following  and 
treating  a  syphilitic  for  several  years  despite  the  dis- 
appearance of  symptoms  is  still  a  good  rule. 


346  TREATMENT 


The  results  of  systematic,  intensive,  intravenous 
salvarsan  therapy  in  atypical  neurosyphilis  (cases 
not  certainly  paretic,  tabetic  or  the  common  types 
of  meningovascular  neurosyphilis)  may  be  in  our 
experience  as  good  as  the  results  of  treatment  hi 
common  meningovascular  cases:  example. 


Case  105.  Henri  Lepere,  a  machinist,  48  years  of  age, 
came  voluntarily  to  the  Psychopathic  Hospital  for  a  grad- 
ually failing  memory  and  inability  to  work.  He  had  had 
indigestion  for  four  years  (epigastric  distress,  nausea,  no 
vomiting).  He  was  still  suffering  from  epigastric  distress 
and  from  headaches.  At  times  he  had  had  difficulty  in 
walking. 

Physically,  Lepere  looked  older  than  he  was;  he  was  very 
poorly  developed  and  nourished,  and  seemed  very  weak. 
There  was  a  slight  visceroptosis. 

Neurologically,  there  was  considerable  speech  defect,  par- 
ticularly well  marked  in  test  phrases.  The  pupils  were 
contracted  and  gave  the  Argyll-Robertson  reaction.  Neuro- 
logically there  were  no  other  signs. 

Mentally,  there  was  a  depression  with  worry;  but  it  was 
a  question  whether  these  phenomena  were  not  entirely 
natural.  The  special  complaint  was  of  failing  memory. 

The  Argyll-Robertson  pupil  also  prima  facie  signifies 
neurosyphilis.  Lepere,  in  fact,  admitted  syphilitic  infection 
at  23.  The  gastric  symptoms  at  once  suggested  tabes.  The 
knee-jerks  and  ankle-jerks  were,  to  be  sure,  preserved;  how- 
ever, this  is  not  very  unusual  in  tabes.  The  amnesia  and 
aphasia  naturally  suggested  paresis.  Without  resort  to 
laboratory  findings,  accordingly,  the  diagnosis  of  tabo- 
paretic  neurosyphilis  ("taboparesis")  was  suggested. 

The  serum  W.  R.  proved  positive,  but  the  spinal  fluid  W.  R. 
very  slightly  so  (yielding  only  moderate  reaction  with  i  cc., 
o.7  and  0.5  cc.,  and  a  negative  reaction  with  0.3  and  O.I  cc.). 


TREATMENT  347 


EFFECT   OF  EARLY  TREATMENT   ON  THE 
DEVELOPMENT   OF  NEUROSYPHILIS 

TOTAL  CASES 4134 

DEVELOPED  GENERAL  PARESIS 198  -   4.8% 

DEVELOPED  TABES  DORSALIS 113  =    2.7% 

DEVELOPED  CEREBROSPINAL  SYPHILIS. ...  132  =    3.2% 


443  -  10.5% 


EFFECT  OF  TREATMENT 


None 

NUMBER  OF  CASES...  ioo  134  924 

DEVELOPED  G.P  ......  25-25%  31=23.1%  30  =    3-2% 

DEVELOPED  TABES...  ii  =11%  16  =  11.9%  25=    2.7% 

DEVELOPED  C.S.S  ......  3=3%  21  =  15.6%  71  =    7-6% 


Poorly  treated  Better  treated 

1880-84  1895-99 

NUMBER  OF  CASES. .  .617  H39 

DEVELOPED  G.P 60=9.7%  37-3-2% 

DEVELOPED  TABES...  22  =3.5%  16  -    1.4% 

DEVELOPED  C.S.S 15  =  2 .4%  28  =    2 .4% 

MATTAUSCHEK  AND  PILCZ 
CHART  24 


348  TREATMENT 

Globulin  was  moderate,  and  albumin  was  found  in  only 
moderate  excess.  There  were  21  cells  per  cmm.  in  the  spinal 
fluid.  The  gold  sol  reaction  was  that  which  we  regard  as 
typical  of  syphilis  or  tabes.  If  we  were  to  rely  upon  the 
weakness  of  the  fluid  W.  R.  and  the  nature  of  the  gold  sol 
reaction,  we  should  be  inclined  to  favor  the  diagnosis  of 
DIFFUSE  NEUROSYPHILIS  ("  cerebrospinal  syphilis  ")  rather 
than  resort  to  the  diagnosis  of  paretic  neurosyphilis. 

Salvarsan  treatment  was  attended  by  the  rapid  disappear- 
ance of  headaches  and  gastric  symptoms  and  by  a  rapid 
gain  in  weight  and  feeling  of  well-being.  Salvarsan  was 
continued  twice  a  week  for  two  months,  whereupon  Lepere 
returned  to  work.  He  has  been  successfully  at  work  now  for 
seven  months  without  return  of  symptoms.  Four  months 
after  beginning  of  treatment,  the  spinal  fluid  was  examined 
and  found  entirely  negative.  Nevertheless,  the  serum  W.  R. 
has  remained  positive  despite  eight  months  of  salvarsan  treat- 
ment. 


What  is  the  meaning  of  the  titrations  in  the  spinal  fluid 
Wassermann  reaction?  When  Plaut  originally  applied 
the  Wassermann  reaction  to  spinal  fluids,  he  used  0.2  of 
a  cc.  of  spinal  fluid.  With  this  amount  of  fluid  he  found 
that  cases  of  general  paresis  gave  a  positive  reaction 
in  about  100%  of  the  cases  while  this  positive  reaction 
was  only  given  by  40  to  60%  of  the  cases  of  cerebro- 
spinal syphilis  and  tabes  dorsalis,  hence  he  promul- 
gated a  differential  point  that  a  negative  reaction  in 
spinal  fluid  indicated  that  the  case  was  not  general 
paresis.  Hauptmann  later  showed  that  if  I  cc.  of  spinal 
fluid  were  used,  a  positive  reaction  would  occur  in 
practically  100%  of  the  cases  of  general  paresis,  cere- 
brospinal syphilis  and  tabes.  Therefore,  at  present, 
we  use  the  different  titers  of  spinal  fluid  from  which  we 
draw  the  following  conclusions:  If  the  reaction  in  the 
untreated  case  is  negative  with  o.l  and  0.3  of  a  cc.  and 
positive  with  the  0.5,  0.7  and  I  cc.  dilutions  as  in  the 
case  of  Lepere,  we  are  probably  dealing  with  non- 
paretic  neurosyphilis.  With  this  method  of  titration 
we  are  also  better  able  to  watch  the  progress  of  treat- 
ment as  the  dilutions  of  O.I  and  0.3  cc.  become  negative 
first. 


TREATMENT  349 

2.  How  soon  can  one  expect  improvement  after  commence- 

ment of  salvarsan  therapy  in  cases  of  diffuse  neuro- 
syphilis?  The  time  relation  of  results  in  treatment  va- 
ries with  each  individual  case.  In  the  case  of  Lepere 
gastric  symptoms  that  had  been  present  for  a  number 
of  months  disappeared  as  if  by  magic  after  the  first 
injection  of  salvarsan.  As  a  rule,  it  is  true  that  the 
more  acute  the  symptoms  the  quicker  their  disap- 
pearance but  this  does  not  hold  for  all  cases,  as  in  this 
particular  instance  the  long  standing  symptoms  dis- 
appeared very  rapidly.  The  symptoms  often  disappear 
very  much  more  rapidly  than  the  laboratory  tests 
change. 

3.  How  can  the  mental  symptoms  (depression  and  failing 

memory)  of  which  patient  complained  be  explained? 
In  the  first  place,  as  has  been  stated,  it  is  doubtful  if 
these  are  more  than  subjective  and  the  result  of  the 
patient's  feeling  of  discomfort  and  pain.  However,  it 
is  also  possible  that  there  may  be  intracranial  involve- 
ment of  the  meninges  or  of  the  brain  itself.  And,  if 
such  were  the  case,  the  improvement  might  be  the 
result  of  the  treatment. 


350  TREATMENT 


The  Argyll-Robertson  pupil  should  not  be  used  as 
a  basis  for  a  necessarily  bad  prognosis  if  treat- 
ment can  be  given. 


Case  1 06.  Frederick  Stone  was  a  business  man  of  large 
interests.  He  had  been  in  the  hands  of  physicians  for  several 
years  for  a  variety  of  disorders  such  as  renal,  respiratory, 
cardio-vascular,  and  so  on.  No  suspicion  of  syphilis  had 
apparently  been  uttered  by  the  physicians  despite  the  fact 
that  Mr.  Stone  readily  stated  that  he  had  had  a  chancre 
thirty  years  before,  and  that  he  had  received  several  years' 
treatment  of  mercury  and  potassium  iodid  by  mouth. 

It  appeared  that  a  few  years  ago  he  had  begun  to  have 
trouble  with  his  nose,  which  was  cauterized  and  operatively 
interfered  with  without  satisfactory  results.  This  nasal 
condition  had  later  been  diagnosticated  as  gummatous,  and 
had  improved  considerably  under  a  mild  antisyphilitic 
treatment.  However,  this  nasal  condition  had  been  con- 
sidered and  treated  quite  separately  from  the  remainder  of 
Mr.  Stone's  troubles. 

What  brought  him  to  attention  was  a  sudden  diplopia  with 
ptosis.  There  was  a  paralysis  of  the  external  rectus  of  the 
left  eye,  as  well  as  a  drooping  of  the  lid  on  this  side.  The 
left  eye  was  much  inflamed.  The  diplopia  greatly  bothered 
the  patient,  and  there  was  also  considerable  pain  in  the  left 
frontal  region,  confined  chiefly  to  the  distribution  of  the  first 
division  of  the  trigeminal  nerve.  According  to  the  patient 
this  headache  was  periodic.  There  was  considerable  tender- 
ness to  pinprick  over  the  area  and  a  diminution  of  sensory 
discrimination  of  fine  touch.  Both  the  pupils  failed  to  react 
to  light. 

The  remainder  of  the  neurological  symptomatic  examina- 
tion was  surprisingly  clear  of  disorder,  nor  was  there  anything 
in  the  history  suggestive  of  tabes.  There  was  ozena  as 
well  as  evidence  of  the  operative  work  upon  nares  and  throat. 
Possibly  the  arteries  were  slightly  hardened;  blood  pressure 


TREATMENT 


PARETIC  NETTROSYPHILIS 

(GENERAL  PARESIS) 

Cases  systematically  treated  50 

CLINICAL  REMISSIONS  34  68% 

C.S.F.  ALTERED  TO  NEGATIVE      4  8% 

C.S.F.  ALTERED  TO  WEAKER  16  32% 

C.S.F.  UNALTERED  14 


CLINICALLY  UNIMPROVED  16  32% 

C.S.F.  WEAKER  7  I4% 

C.S.F.  UNALTERED  9  18% 


MASSACHUSETTS  COMMISSION  ON  MENTAL  DISEASES 

November,  1916 


CHART  25 


352 


TREATMENT 


was  165  systolic.  There  was  a  large  trace  of  albumin,  and 
there  were  numerous  hyalin  casts  in  the  urine. 

Mentally,  there  was  a  degree  of  depression  and  worry 
hardly  out  of  keeping  with  the  general  situation.  Despite 
the  preservation  of  memory,  Mr.  Stone  failed  to  do  rather 
simple  arithmetical  calculations;  this  was  the  more  re- 
markable as  in  his  business  he  had  to  handle  figures  a  great 
deal  and  had  been  doing  so  until  recently.  There  was  a 
slight  tremor  in  his  writing,  as  well  as  a  certain  difficulty  in 
enunciating  test  phrases.  Insomnia,  irritability,  and  a  feel- 
ing of  nervousness  and  of  being  tired  out,  completed  the 
picture. 

A  suggestion  for  diagnosis  would  be  classically  offered 
by  the  Argyll- Robertson  pupils.  Should  not  a  patient  with 
the  Argyll- Robertson  pupils  have  either  tabes  or  paresis? 
However,  in  favor  of  tabes,  besides  the  pupil,  are  to  be  counted 
merely  the  troubles  with  the  eyes.  In  the  direction  of  paresis 
we  have  to  consider  speech  defect,  to  say  nothing  of  less  defi- 
nite symptoms  such  as  insomnia  and  increased  irritability. 

We  are  inclined  to  think,  however,  that  the  disease  in  this 
case  is  meningovascular.  This  diagnosis  is  suggested  by  the 
cranial  nerve  palsies  and  by  the  headache.  Headache  is 
much  more  rarely  a  phenomenon  in  the  paretic  type  of  neuro- 
syphilis  than  in  the  meningovascular  type. 

In  point  of  fact,  the  spinal  fluid  phenomena  bore  out  the 
diagnosis  of  MENINGOVASCULAR  NEUROSYPHILIS  inasmuch  as 
the  globulin,  albumin,  cellular  content,  gold  sol,  and  W.  R.'s 
were  all  weakly  positive. 

I.  How  far  can  we  regard  the  cardiorenal  defects  as  syphi- 
litic? Perhaps  we  may  do  so  on  the  general  principle 
of  parsimony  in  scientific  interpretation. 

The  diagnostic  lumbar  puncture  led  to  an  extremely 
severe  exacerbation  of  the  pains  on  the  left  side  of  the  head. 
In  fact,  these  pains  could  not  be  held  in  check  by  the  ex- 
hibition of  pyramidon.  Mr.  Stone  regarded  the  pain  as  due 
to  the  lumbar  puncture.  However,  there  was  no  improve- 
ment in  the  pain  in  the  prone  position,  —  a  feature  charac- 
teristic of  lumbar  puncture  pains.  Upon  administration  of 


TREATMENT  353 

salvarsan,  this  local  pain  rapidly  disappeared.  In  fact,  there 
was  a  startling  improvement;  the  ocular  palsies  disappeared 
in  a  few  weeks,  although  these  palsies  had  been  present 
for  several  months  before  the  administration  of  salvarsan. 
The  blood  pressure  was  reduced ;  the  urine  became  negative. 
Perhaps  the  most  startling  feature  of  all  (although  of  this  we 
are  not  sure)  was  that  the  patient  states  he  was  accepted  by 
a  life  insurance  company  although  he  had  been  twice  refused 
previously. 

Note  in  this  case  the  3O-year  interval  between  infection 
and  generalized  neurosyphilitic  involvement.  Note  also 
the  amenability  of  the  process  despite  this  duration.  We 
are  perhaps  entitled  also  to  note  that  a  neurological  exami- 
nation careful  enough  to  detect  an  Argyll-Robertson  pupil 
should  have  been  made  by  a  number  of  examiners  long 
before  the  particular  crisis  which  we  have  sketched.  It  is 
also  permissible  to  note  that  the  rhinological  work  should  not 
have  been  carried  out  independently  of  all  other  medical 
work. 


2.  What  are  the  untoward  results  of  lumbar  puncture? 
It  is  true  that  there  is  always  a  possibility  of  setting  up 
a  septic  meningitis  by  lumbar  puncture,  but  this  is  a 
very  remote  possibility  and  with  any  reasonable  care 
it  is  not  to  be  considered.  Lumbar  puncture  also  has 
a  considerable  danger  in  cases  of  increased  intracranial 
pressure.  In  cases  of  brain  tumor  where  the  tumor  is 
located  in  the  posterior  fossa,  sudden  death  may  occur 
from  withdrawal  of  spinal  fluid.  This  is  supposed  to 
be  due  to  the  medulla  being  pressed  down  into  the 
foramen  magnum  and  causing  paralysis  of  respiration. 
Therefore  lumbar  puncture  should  never  be  performed 
except  with  the  greatest  caution  in  a  case  in  which 
brain  tumor  is  suspected. 

However,  aside  from  these  remote  serious  conse- 
quences which  play  very  little  r61e  in  the  ordinary  pro- 
cedure of  lumbar  puncture,  certain  unpleasant  symp- 
toms do  frequently  arise.  These  symptoms  are  chiefly 
headache  and  nausea,  but,  however,  may  go  as  far  as 
vomiting.  These  symptoms  occur  almost  entirely  in 
the  cases  in  which  there  is  no  abnormal  condition  pro- 
ducing increased  spinal  fluid  pressure.  Such  unpleasant 


354 


TREATMENT 


symptoms  may  last  as  long  as  four  or  five  days;  as  a 
rule,  however,  last  only  for  a  period  of  a  day  or  two. 

3.  What  is  the  treatment  of  discomfort  following  lumbar 

puncture?  It  is  a  rule  well  worth  observing  that  the 
patient  after  lumbar  puncture  should  remain  flat  on  his 
back  without  a  pillow  for  24  hours  in  order  to  avoid  any 
unpleasant  symptoms.  If  any  symptoms  do  occur, 
it  will  be  almost  certainly  when  the  patient  arises,  and 
in  nearly  every  instance  they  will  be  overcome  if  the 
patient  again  assumes  the  prone  position.  Raising 
the  foot  of  the  bed  so  as  to  lower  the  head  also  helps. 
Veronal  or  bromides  may  be  given  but  as  a  rule  are  not 
very  satisfactory. 

4.  How  permanent  is  the  improvement  obtained  in  the 

case  of  Mr.  Stone  likely  to  be?  As  a  matter  of  fact, 
the  patient  discontinued  treatment  as  soon  as  he  felt 
well  again,  but  after  two  months  the  pain  returned  to 
be  again  quickly  dispelled  by  salvarsan.  This  im- 
provement must  be  considered  as  only  temporary. 
Under  continued  treatment  there  may  be  no  further 
relapse.  There  is,  however,  evidence  that  much  dam- 
age has  been  done  to  the  body  by  the  spirochetes, 
much  of  which  is  irreparable.  It  is  even  possible  that 
further  disintegration  might  occur  even  while  under- 
going treatment.  Still  treatment  offers  much  in  such 
a  case  and  is  to  be  highly  recommended. 


TREATMENT  355 


In  DIFFUSE  NEURO  SYPHILIS,  rendering  the 
spinal  fluid  negative  by  treatment  may  mean 
neither  cure  nor  disappearance  of  symptoms. 


Case  107.  Greta  Meyer,  a  widow,  51  years  of  age,  came 
voluntarily  to  the  hospital,  seeking  medical  aid  for  a  marked 
depression.  She  was  also  suffering  from  a  right  hemiplegia. 
It  appeared,  according  to  Mrs.  Meyer,  that  she  was  married 
at  1 6,  and  lived  with  her  husband  until  29,  whereupon  she 
left  him  on  account  of  his  alcoholism,  his  abuse  of  her,  and 
the  discovery  through  his  physician  that  he  was  suffering  from 
venereal  disease.  She  had  had  two  healthy  children  and 
there  never  had  been  miscarriages  or  stillbirths.  Six  years 
after  the  separation,  namely  at  35  years  of  age,  and  16  years 
before  resort  to  the  Psychopathic  Hospital,  Mrs.  Meyer 
developed  certain  red  areas  on  her  hand,  and  learned  at  a 
hospital  that  these  were  due  to  syphilis.  She  kept  up 
treatment  for  these  lesions  for  a  year,  until  she  seemed  per- 
fectly well. 

She  had,  in  fact,  remained  perfectly  well  for  some  14  years, 
until  at  49,  a  small  tumor  had  appeared  on  the  right  side  of 
the  forehead,  near  the  hair  line.  This  tumor  was  firm  and 
not  sore.  Medical  treatment  reduced  it,  leaving,  however, 
a  depression  in  the  bone.  One  day,  about  a  month  after  the 
appearance  of  the  tumor,  the  patient  lay  down  for  a  nap, 
and  upon  awaking  found  she  could  only  with  difficulty  move 
her  right  arm  and  leg.  Her  face  was  not  affected;  she  was 
not  in  pain ;  and  there  was  no  disorder  of  speech.  In  a  few 
days  she  got  much  better  and  she  had  been  improving 
for  some  time  past  through  the  administration  of  further 
medicine. 

However,  since  the  onset  of  the  hemiplegia  Mrs.  Meyer 
had  been  very  despondent.  There  had  been  ups  and  downs 
but  she  had  rarely  felt  well.  The  depression  was  a  mild  one 
and  in  point  of  fact  may  perhaps  be  regarded  as  non-psycho- 
pathic, since  at  her  age  with  her  disability,  there  might  well  be 


356  TREATMENT 


METHODS   OF  TREATMENT 

I.    BY  MOUTH. 

1.  MERCURY 

2.  IODIDES 

3.  ARSENIC. 

.II.     INTRAMUSCULAR  INJECTIONS 

1.  MERCURY 

2.  SALVARSAN,     NEOSALVARSAN,     OTHER     ARSENIC! 

PREPARATIONS 

3.  SODIUM   NUCLEINATE 

4.  ANTIMONY 

III.  INTRAVENOUS 

1.  MERCURY 

2.  MERCURIALIZED  SERUM 

3.  SALVARSAN,  NEOSALVARSAN,  ARSENIC 

4.  IODIDES 

IV.  SPINAL  INTRADURAL 

1.  SALVARSANIZED  SERUM   (!N  Vivo— SWIFT-ELLIS) 

2.  SALVARSANIZED      SERUM      (!N  VITRO  —  MARINESCO- 

OGILVIE) 

3.  MERCURIALIZED  SERUM   (BYRNES) 

V.  CEREBRAL  SUBDURAL  AND  INTRAVENTRICULAR 

1.  SALVARSANIZED  SERUM   (!N  Vivo) 

2.  SALVARSANIZED  SERUM   (!N  VITRO) 

3.  MERCURIALIZED  SERUM 


CHART  26 


TREATMENT  357 

a  degree  of  sadness  and  unhappiness  concerning  the  future. 
Mentally,  there  was  no  other  disorder  of  note,  and  in  particu- 
lar no  disorder  of  memory. 

Physically,  the  patient  showed  a  right-sided  hemiplegia 
with  excessive  right  knee-jerk,  but  without  Babinski  or  other 
abnormal  reflex  phenomena.  The  extra-ocular  movements 
were  somewhat  restricted  in  range  but  there  was  neither 
strabismus  nor  nystagmus. 

The  question  arose  whether  the  hemiplegia  was  of  hemor- 
rhagic  or  thrombotic  origin.  After  all,  at  51  years,  hemiplegia 
is  rather  unlikely  to  be  of  a  non-syphilitic  arteriosclerotic 
origin;  moreover,  we  had  a  clear  history  of  syphilis.  The 
serum  W.  R.  proved  positive  as  well  as  the  spinal  fluid  W.  R. 
The  finding  of  77  cells  per  cmm.,  excess  albumin,  and  positive 
globulin  test,  taken  in  connection  with  the  entire  picture 
seems  to  warrant  a  diagnosis  of  CEREBROSPINAL  SYPHILIS" 
If  we  proceed  on  statistical  grounds,  it  might  be  regarded  as 
more  probable  that  the  hemiplegia  is  THROMBOTIC  in  origin 
rather  than  hemorrhagic.  It  appears  that  syphilitic  cerebral 
thrombosis  rather  characteristically  occurs  without  prelimi- 
nary symptoms,  despite  the  fact  that  many  cases  do  show 
headache,  dizziness,  and  restlessness  as  prodromal  symptoms. 

I.  What  is  the  treatment  indicated  in  the  case  of  Mrs. 
Meyer? 

It  would  appear  that  little  or  nothing  can  be  done 
for  the  hemiplegia  unless  the  claims  of  Franz  with 
respect  to  reestablishment  of  a  degree  of  function  in 
certain  hemiplegics  are  substantiated.  However,  the 
indication  of  meningitic  process  as  shown  by  the  spinal 
fluid,  suggests  that  the  case  is  not  a  purely  vascular  one 
but  may  be  regarded  as  meningovascular.  (Possibly, 
also,  we  should  regard  the  left  frontal  depression  and 
scar  as  indicative  of  a  non-parenchymatous  and  non- 
vascular  process.)  Accordingly,  antisyphilitic  treat- 
ment should  be  theoretically  of  some  value. 

In  point  of  fact,  the  patient  was  given  injections  of 
mercury  salicylate,  mercury  by  mouth,  and  potassium 
iodid.  Her  psychopathic  depression  under  this  treat- 
ment, supported  by  proper  hygiene  and  rest,  dimin- 
ished. However,  six  months  later,  the  patient  slipped 
on  a  wet  floor  and  fell.  Though  the  impact  seemed 


358  TREATMENT 

hardly  sufficient  to  cause  a  fracture,  the  pelvis  was 
somewhat  severely  fractured.  Very  probably  there 
was  a  syphilitic  rarefaction  of  the  bone.  Six  months 
later  the  patient's  depression  was  still  in  evidence, 
though  somewhat  less  than  upon  admission.  The 
blood  serum  remained  positive  but  the  spinal  fluid  had 
become  entirely  negative,  both  in  respect  to  the  W.  R. 
and  in  respect  to  the  other  findings. 

2.  How  may  one  explain  the  continuance  of  the  depression 

after  the  spinal  fluid  had  become  entirely  negative  under 
treatment?  It  may  be  that  while  the  active  process 
had  been  stopped,  as  seems  probable  from  the  negative 
spinal  fluid,  that  a  permanent  destruction  of  brain 
tissue  may  account  for  the  depression.  We  recognize 
this  readily  in  instances  of  vascular  disturbance  where 
(as  also  in  this  case)  the  active  process  being  stopped,  a 
residual  defect  remains. 

3.  Should  treatment  have  been  discontinued  on  reduction  of 

the  gumma?  It  cannot  be  too  often  emphasized  that 
the  disappearance  of  symptoms  in  cases  of  syphilis  can 
not  be  considered  as  evidence  of  cure.  The  neurologist 
and  psychiatrist  see  only  too  often  cases  of  neurosy- 
philis  occurring  in  patients  who  have  been  declared 
cured  at  some  time  previous  because  the  symptoms  then 
present  had  cleared  up  and  remain  in  abeyance  for 
years. 


TREATMENT  359 


Contrary  to  various  warnings,  arteriosclerosis  by 
no  means  absolutely  contraindicates  intensive 
salvarsan  therapy. 


Case  1 08.  Victor  Friedberg,  42  years  of  age,  gave  the  fol- 
lowing history.  He  acquired  syphilis  at  22  years.  He  had 
"  adequate  "  medical  treatment  for  two  years  with  inunc- 
tions of  mercury  and  mercury  by  mouth  and  potassium  iodid. 
The  only  secondary  symptoms  were  skin  lesions  of  the  legs; 
these  disappeared  upon  treatment.  Married,  Friedberg  has 
one  child,  apparently  normal.  There  had  been  no  miscar- 
riages or  stillbirths. 

At  about  34  years,  there  began  to  be  shooting  pains  in  the 
legs,  occurring  at  first  about  once  in  three  months,  but  later 
much  more  frequently.  These  pains  were  severe,  lightning 
in  character,  lasting  several  days  at  a  time,  at  which  period 
his  head  would  feel  heavy;  but  there  were  no  disturbances, 
crises,  or  difficulty  in  locomotion. 

At  36  years  of  age,  Friedberg  waked  up  with  pain  one  night, 
and  found  he  was  unable  to  move  his  left  leg  or  hand,  and  he 
felt  his  mouth  drawn  to  the  left.  Upon  trying  to  get  out  of 
bed,  he  fell  to  the  floor.  In  five  hours,  however,  he  was 
entirely  recovered,  able  to  get  up  and  walk  about,  and  to  use 
his  left  arm  quite  normally.  He  went  to  sleep,  but  upon 
waking  up  after  an  hour,  discovered  that  his  left  side  was 
again  paralyzed.  After  two  weeks  in  a  hospital,  he  was  able 
to  walk  with  a  crutch.  The  arm  remained  helpless  for  about 
a  year.  Both  arm  and  leg  improved  slowly  for  two  years, 
after  which  time  his  condition  had  remained  stationary. 
For  four  years  past,  there  had  been  no  more  pain,  but  at  42  - 
about  two  years  before  admission  —  the  pains  returned  in  his 
legs,  back,  and  side.  At  that  time  he  received  four  injections 
of  salvarsan,  mercury  tablets,  and  potassium  iodid.  Three 
weeks  before  admission  to  the  hospital,  Friedberg  again 
began  having  headaches,  very  much  worse  than  formerly. 
At  first  these  headaches  were  frontal,  then  occipital,  and  there 


360  TREATMENT 

was  a  feeling  as  if  something  were  growling  inside  of  the  head. 
There  was  a  feeling  of  pressure  in  front  on  the  head  and  at  the 
base  of  the  nose. 

Physically,  Friedberg  appeared  somewhat  older  than  his 
assigned  age.  There  was  a  degree  of  general  peripheral 
arteriosclerosis,  but  in  general  the  physical  examination  was 
negative.  Neurologically,  there  was  a  left  hemiplegia  with 
appropriate  increase  of  the  reflexes  on  that  side,  spasticity, 
Babinski  reflex,  and  an  Oppenheim ;  the  pupils  reacted  prop- 
erly ;  there  was  no  Romberg  reaction. 

Mentally,  Friedberg  was  entirely  negative. 

The  W.  R.  of  the  blood  serum  was  doubtful,  as  was  that  of 
the  spinal  fluid.  There  were  but  two  cells  per  cmm.  and  there 
was  neither  globulin  nor  excess  albumin  in  the  spinal  fluid. 

The  differential  diagnosis  might  lie  between  cerebral 
hemorrhage  and  syphilitic  thrombosis.  Thrombosis  is  much 
more  common  as  a  result  of  syphilis  than  is  hemorrhage. 
The  occurrence  of  the  thrombosis  during  sleep  without  pre- 
monitory symptoms  is  also  characteristic  in  syphilis.  Pos- 
sibly there  was  a  low-grade  spinal  meningitis  at  the  bottom  of 
the  lancinating  pains.  Whether  the  headache  is  an  arterio- 
sclerotic  effect  or  due  to  a  meningitis  not  shown  in  the 
cerebrospinal  fluid  is  doubtful.  However,  the  absence  of  in- 
flammatory products  in  the  cerebrospinal  fluid  rather  indicates 
that  the  headache  is  of  arteriosclerotic  origin.  Autopsies, 
however,  warn  us  that  we  may  have  a  localized  meningitis 
in  various  parts  of  the  cranial  cavity  without  the  determina- 
tion of  any  inflammatory  products  in  the  spinal  fluid. 

I.  How  shall  we  explain  the  doubtful  (slightly  positive) 
W.  R.  in  the  spinal  fluid  if  the  case  is  one  of  VASCULAR 
BRAIN  SYPHILIS?  The  finding  is  not  unusual  in  these 
cases.  The  W.  R.  producing  body  is  recognized  to  be 
of  a  separate  nature  from  the  globulin  and  albumin 
bodies,  and  is  probably  also  separate  from  the  gold  sol 
reaction  producing  bodies. 

Treatment:  The  theory  of  treatment  is  that  any  spi- 
rochetes  that  may  be  still  active  in  the  body  should  be  de- 
stroyed. Accordingly,  although  salvarsan  can  certainly  have 


TREATMENT  361 

no  effect  in  reproducing  nerve  tissue,  it  nevertheless  seems 
indicated.  It  is  frequently  stated,  however,  that  salvarsan 
is  dangerous  in  cases  of  this  group.  We  have  not  found  this 
statement  correct.  In  this  case,  there  was  a  symptomatic 
improvement,  as  far  as  pain  and  discomfort  went,  under 
salvarsan  and  iodids. 

2.  What  precautions  should  be  taken  in  intensive  salvarsan 

treatment  of  syphilitic  arteriosclerosis?  Treatment 
should  be  begun  with  very  small  doses  of  salvarsan, 
that  is,  about  o.i  of  a  gram  and  then  the  amount  slowly 
increased.  The  injection  should  be  given  slowly  so  as 
not  to  put  too  great  a  load  upon  the  cardiovascular 
system. 

3.  What  r61e  does  the  mental  attitude  of  the  patient  play  in 

a  case  like  that  of  Friedberg?  It  was  quite  evident 
that  Friedberg  was  neurotic  and  that  he  had  a  syphil- 
ophobia.  Consequently  some  of  the  symptomatic  im- 
provement may  have  been  more  results  of  assurances 
offered  by  the  physician  and  knowledge  that  he  was 
being  treated,  than  results  of  salvarsan.  In  some  cases 
mental  anguish  suffered  by  the  patient  is  of  more 
importance  than  the  actual  symptoms  of  the  disease 
and  this  point  must  be  always  borne  in  mind  in  handling 
syphilitic  patients. 


362  TREATMENT 


Symptoms  of  intracranial  pressure  cured  by  anti- 
syphilitic  treatment. 


Case  109.  Mrs.  Annie  Rivers,  a  housewife  36  years  of  age, 
sought  advice  and  treatment  for  severe  convulsions  which 
she  had  had  during  a  period  of  several  weeks.  She  left  the 
hospital  before  being  properly  examined,  and  had  several 
more  convulsions,  after  which  she  was  brought  back  in  a  state 
of  marked  confusion.  The  confusion  shortly  disappeared 
almost  completely,  and  a  good  history  was  obtained. 

It  appears  that  the  patient  led  a  normal  life  and  had  had 
six  children,  the  last  of  whom  was  born  about  four  months 
before  her  coming  to  the  hospital.  The  first  symptoms 
appeared  about  a  month  after  the  birth  of  the  child,  when, 
one  afternoon,  Mrs.  Rivers  suddenly  fell  unconscious  while 
ironing.  She  remained  unconscious  for  nearly  three  hours. 
During  this  attack  there  were  no  convulsive  movements  or 
tongue  biting;  and  after  the  spell,  she  felt  neither  lame  nor 
sore,  but  merely  tired.  This  was  Mrs.  Rivers'  statement; 
but  her  daughter  stated  that  the  patient  really  did  have  con- 
vulsive movements.  A  week  later  came  a  second  convul- 
sion, followed  by  daze  and  stupor.  This  second  attack 
lasted  two  hours. 

About  a  week  before  entrance,  the  patient  had  remained 
in  bed  on  account  of  dull  grinding  pain  in  the  left  side  of  the 
head,  below  the  ear,  and  upon  this  day  the  patient  vomited 
twice.  In  addition  to  the  dull  grinding  pain,  there  were 
pains  referred  to  the  ear  itself  and  to  the  left  side  of  the  head, 
especially  over  the  left  eye;  there  were  no  pains  on  the  right 
side  of  the  head.  The  next  day  the  patient  was  better,  but 
the  day  thereafter  again  remained  in  bed.  The  only  other 
symptoms  were  cold  feelings  at  times  and  bright  spots  in  the 
field  of  vision. 

No  mental  symptoms  were  observed  in  Mrs.  Rivers  except 
a  bit  of  depression  after  her  hasty  retreat  from  the  hospital 
the  first  time.  Upon  her  second  admission,  however,  after 


TREATMENT  363 


UNTOWARD  SYMPTOMS   OF  THERAPEUTIC 
AGENTS 

A.  SALVARSAN 

CYANOSIS  MALAISE 

RAPID  PULSE 

PERSPIRATION 

RESPIRATORY  DIFFICULTIES 

FEVER 

NAUSEA,  VOMITING,   DIARRHOEA 

DERMATOSES 

EDEMA 

KIDNEY  IRRITATION 

LIVER  IRRITATION 

INTENSIFICATION  OF  SYMPTOMS 

COLLAPSE 

B.  MERCURY 
SALIVATION 

FETID   BREATH 

EXCESS  FLOW  OF  SALIVA 

TENDERNESS  OF  TEETH  —  LOOSENING  AND   FALL- 
ING OUT 

SPONGY  GUMS  — EROSION 

METALLIC  TASTE 

NECROSIS  OF  BONES  OF  JAW 

SORENESS  OF  PAROTIC  AND   MAXILLARY  GLANDS 

SWELLING  AND  EROSION  OF  TONGUE  AND  MUCOUS 

MEMBRANES 

GASTRO-INTESTINAL  SYMPTOMS 
ANEMIA 

PAIN   IN  JOINTS 
NEPHRITIS 

C.  IODINE 

SKIN  LESIONS 

METALLIC  TASTE 

SALIVATION 

CORYZA 

URTICARIA  (EVEN  TO  GRADE  OF  ANGIONEUROTIC  EDEMA) 

PAINS 

CONSTIPATION 

INVOLVEMENT  OF  JOINTS 

FEVER 

SOFTENING  AND  BLEEDING  OF  GUMS 

EROSION  OF  MUCOUS  MEMBRANES 

GASTRO-INTESTINAL  SYMPTOMS 

ANOREXIA 

WEAKNESS  CHART  27 


364  TREATMENT 

a  week  or  ten  days'  residence,  apathy  developed  together 
with  considerable  amnesia  for  the  same  facts  she  had  quite 
readily  remembered  a  few  days  previously.  Along  with  the 
apathy  and  amnesia  developed  considerable  headache;  and 
there  were  attacks  of  vomiting. 

On  the  physical  side,  it  is  interesting  to  note  that  the  oph- 
thalmoscopic  examination  upon  Mrs.  Rivers'  first  admission 
to  the  hospital  was  entirely  negative,  whereas  a  week  later, 
pronounced  difficulty  with  vision  appeared  so  that  in  a  few 
days  she  was  able  to  make  out  only  very  large  type.  The 
fundi  now  showed  hazy  and  indistinct  disc  outlines,  with 
small  yellowish  areas  of  fatty  degeneration  above  the  disc, 
reduction  of  arterial  calibre,  and  dilated  and  somewhat  tor- 
tuous veins  (no  projection  of  papillae),  so  that  the  ophthal- 
mological  diagnosis  was  chronic  neuritis. 

The  physical  examination  otherwise  was  mostly  negative. 
The  skin  presented  irregular  areas  covered  with  silvery  scales 
over  the  arms  and  chest,  back,  abdomen,  and  legs  (the  patient 
had  had  psoriasis  several  years  before).  Both  pupils  reacted 
to  light  and  distance,  though  the  right  was  slightly  larger 
than  the  left  and  somewhat  irregular.  There  was  a  slight 
tremor  of  the  tongue  and  extended  fingers.  The  reflexes 
were  active,  especially  the  knee-jerks;  no  abdominal  reflexes 
could  be  obtained.  The  serum  W.  R.  was  positive,  but  the 
spinal  fluid  W.  R.  was  negative.  The  spinal  fluid  showed 
but  3  cells  per  cmm.,  but  there  was  a  positive  globulin  test 
and  an  excess  of  albumin. 

Diagnosis:  After  the  symptoms  had  fully  developed,  it 
became  clear  from  the  optic  neuritis,  headaches,  and  vomiting 
that  a  condition  of  intracranial  pressure  existed.  In  view 
of  the  positive  serum  W.  R.,  it  is  natural  to  conceive  that  the 
agent  producing  the  intracranial  pressure  was  a  gumma. 

It  is,  of  course,  possible  that  a  marked  degree  of  meningitis 
might  be  so  localized  as  to  produce  the  same  symptoms.  The 
diagnostician  would  crave  a  pleocytosis  of  the  spinal  fluid 
if  a  diagnosis  of  meningitis  is  to  be  made;  and  there  was  no 
such  pleocytosis.  On  the  whole,  we  do  not  feel  that  it  is 
possible  to  make  a  diagnosis  either  of  MENINGITIS  or  of 
GUMMA. 


TREATMENT  365 

Treatment:  Treatment,  however,  caused  a  disappearance 
of  all  symptoms.  The  treatment  consisted  of  but  one  injec- 
tion of  0.3  gram  of  salvarsan,  followed  by  a  few  injections  of 
mercury;  whereupon  Mrs.  Rivers  became  much  brighter, 
recovered  her  vision,  lost  her  headaches,  ceased  to  have  con- 
vulsions or  vomiting  spells. 

_I.  Is  salvarsan  contraindicated  in  cases  with  involvement  of 
the  optic  or  auditory  nerves?  Such  a  contraindication 
exists  according  to  prevailing  opinion.  In  this  partic- 
ular case,  a  hemorrhagic  retinitis  occurred  after  the 
injection  of  salvarsan,  but  this  retinitis  disappeared 
along  with  the  other  symptoms.  On  the  whole  we 
believe  that  in  many  cases  of  optic  or  auditory  nerve 
involvement  salvarsan  should  be  used.  However,  one 
should  never  lose  sight  of  the  possibility  of  untoward 
results  and  should  advise  such  treatment  only  when 
other  treatment  seems  inefficient. 


366  TREATMENT 


TABETIC  NEUROSYPHILIS  ("tabes  dorsalis  ») 
may  show  very  marked  improvement  as  a  result 
of  intraspinous  therapy. 


Case  no.  Mr.  McKenzie*  was  a  retired  merchant  of  42 
years  whose  complaint  was  that  he  tired  very  easily,  could  not 
make  his  legs  go  where  he  wished,  was  unsteady  and  felt  a 
numbness  in  his  legs.  These  symptoms  had  been  in  prog- 
ress for  a  few  months  only  when  the  examination  was  made. 
This  disclosed  Argyll-Robertson  pupils,  absent  knee-jerks 
and  ankle-jerks,  Romberg  sign,  unsteady  gait,  moderate 
ataxia  and  dysmetria.  The  W.  R.  was  negative  in  the 
blood  serum  but  positive  in  the  spinal  fluid  with  0.2  cc.,  and 
there  were  107  cells  per  cmm.  With  the  symptoms  and  signs 
it  was  therefore  easy  to  make  the  diagnosis  of  TABETIC 
NEUROSYPHILIS  ("  tabes  dorsalis  "). 

The  patient  was  given  five  intraspinous  injections  of  mer- 
curic chloride  in  blood  serum  (mercurialized  serum)  according 
to  the  method  of  Byrnes.  The  dose  was  o.ooi  gm.  of  mercury. 
Two  weeks  after  the  first  injection  the  cell  count  was  58  cells 
per  cmm.,  the  Wassermann  was  positive  only  with  0.4  cc. 
After  the  fourth  injection  there  were  but  18  cells  and  the 
Wassermann  reaction  was  negative  even  with  i£  cc.  of  spinal 
fluids.  The  symptoms  had  improved  to  such  a  degree  that  the 
patient  had  no  complaint  whatsoever  and  considered  himself 
cured. 

I.  What  are  the  unpleasant  results  of  intraspinous  therapy? 
Frequently  there  is  an  exacerbation  of  symptoms  and 
pain  may  be  quite  severe  after  intraspinous  injections. 
This,  however,  lasts  only  a  short  period,  that  is,  as  a 
rule  less  than  24  hours.  There  may  be  other  symp- 
toms of  cord  irritation  as  retention  of  urine  or  lack  of 
sphincter  control.  A  rise  of  temperature  is  not  unusual. 

*  (This  case  was  furnished  by  Dr.  D.  A.  Haller  from  the 
Peter  Bent  Brigham  Hospital  series.) 


TREATMENT  367 


Treatment  may  alter  the  W.  R.  to  negative  in 
blood  and  spinal  fluid  in  TABES  DORSALIS. 


Case  in.  Ivan  Rokicki  was  a  baker,  43  years  of  age,  who 
came  complaining  of  exceedingly  severe  attacks  of  abdominal 
pain  with  vomiting.  He  described  these  attacks  as  having 
occurred  periodically  for  a  number  of  years,  lasting  sometimes 
as  long  as  a  week,  during  which  time  Rokicki  could  not  eat  or 
get  relief  short  of  large  doses  of  morphine. 

Upon  his  arrival,  Rokicki  was  seen  in  one  of  his  attacks; 
he  was  curled  up  with  excruciating  pain,  and  the  abdomen 
was  rigid,  though  it  was  impossible  to  produce  additional 
pain  by  external  pressure.  There  was  spasmodic  vomiting, 
frequently  followed  by  slight  relief  from  the  pain,  which 
however  shortly  recurred  and  caused  the  patient  to  cry  out  in 
his  suffering.  The  condition  was  controlled  by  opiates  but 
lasted  a  full  week.  The  leucocytes  remained  normal  and 
there  was  no  rise  of  temperature.  The  attack  ceased  spon- 
taneously. 

Save  for  the  pain,  Rokicki's  mental  examination  proved 
entirely  negative.  Physically,  Rokicki  was  fairly  well  devel- 
oped and  nourished.  His  pupils  were  slightly  irregular:  the 
left  markedly  larger  than  the  right;  both  pupils  failed  to 
react  to  light,  and  the  left  pupil  also  failed  to  react  in  ac- 
commodation. There  were  no  other  reflex  disorders  evident 
to  systematic  examination,  nor  was  there  sensory  distur- 
bance or  speech  defect.  The  heart  seemed  somewhat  en- 
larged but  there  were  no  murmurs;  blood  pressure:  systolic 
150;  dia'stolic  no. 

The  correct  symptomatic  diagnosis  in  Rokicki's  case  proved 
to  be  gastric  crises,  and  this  diagnosis  must  perforce  be  the 
first  to  entertain  in  view  of  the  chronicity,  the  periodicity, 
the  non-relation  to  diet,  and  the  spontaneous  cessation  of 
the  seizures.  The  observation  of  Argyll-Robertson  pupils 
was  naturally  held  to  substantiate  the  diagnosis  of  TABES 
DORSALIS. 


368  TREATMENT 

The  possibility  of  abdominal  inflammation  could  be  shortly 
dismissed  on  account  of  the  absence  of  tenderness  (the  rigid- 
ity in  this  case  was  not  accompanied  by  tenderness),  fever, 
and  other  characteristic  signs.  There  was  no  diarrhoea,  such 
as  is  found  in  lead  colic,  and  there  was  no  other  sign  of 
plumbism.  Jaundice  was  absent  and  there  was  no  special 
radiation  of  pain  from  the  abdomen.  One  had  to  think  of 
gastric  ulcer  and  hyperchlorhydia,  and  possibly  malaria  or 
gastroenteritis. 

The  pupillary  reactions  pointed  to  a  syphilitic  condition 
despite  the  fact  that  the  lack  of  reaction  to  accommodation 
(over  and  above  the  Argyll-Robertson  phenomenon)  in  the 
right  pupil  is  not  entirely  typical.  Accordingly,  although 
there  was  no  areflexia,  Romberg  sign,  or  ataxia,  resort  was 
had  to  the  W.  R.  This  however  proved  negative,  in  blood 
and  spinal  fluid;  nor  was  there  any  globulin  or  excess  albu- 
min; there  were  5  cells  to  the  cmm.,  in  the  spinal  fluid. 

We  are  left,  accordingly,  with  characteristic  gastric  crises; 
Argyll-Robertson  pupils,  slightly  irregular;  and  a  somewhat 
enlarged  heart. 

Upon  investigation,  it  appeared,  however,  that  a  year 
before  the  attack  above  described,  the  patient  had  been 
examined  and  both  blood  and  spinal  fluid  found  positive  to 
the  W.  R.  At  that  time,  treatment,  consisting  of  intravenous 
injections  of  salvarsan  and  intraspinous  injections  of  salvarsan- 
ized  serum  (Swift-Ellis),  had  been  instituted.  Whereupon 
the  laboratory  tests  had  become  negative,  as  above  stated, 
and  there  had  been  no  alleviation  of  the  symptoms. 

1.  How  can  Rokicki's  normal  deep  leg  reflexes  be  explained? 

The  abolition  of  the  deep  reflexes  is  of  course  due  to 
lesions  properly  localized.  It  is  probable  that  this 
particular  case  of  tabes  dorsalis  is  more  truly  "  dorsal  " 
than  most  cases ;  for  most  cases  exhibit  lesions  involving 
regions  lower  than  the  dorsal.  Both  in  these  dorsal 
cases  and  in  certain  rare  cases  of  cervical  tabes,  the 
deep  leg  reflexes  are  preserved.  (See  cases  Green  (30) 
and  Halleck  (31).) 

2.  What  is  the  mechanism  by  which  a  characteristic  gastric 

crisis  is  produced?  The  mechanism  is  unknown.  Some 
endeavors  have  been  made  to  meet  gastric  crises  by 


TREATMENT  369 

surgery  of  the  posterior  roots,  on  the  assumption  that 
the  irritation  causing  the  pain  was  located  either  in 
the  posterior  ganglion  or  in  the  passage  of  the  nerve 
through  the  meninges.  In  only  a  few  instances,  how- 
ever, has  the  result  been  what  was  desired.  In  many 
instances  the  gastric  crises  and  pain  continued  uninter- 
rupted and  in  addition  came  discomfort  due  to  the  lack 
of  sensation  in  the  part  supplied  by  the  severed  nerve. 
At  present  this  treatment  is  seldom  carried  out. 
Should  antisyphilitic  treatment  be  continued  in  such  a 
case?  As  far  as  our  present  knowledge  of  syphilis  goes 
one  would  hesitate  to  suggest  further  antisyphilitic 
treatment,  feeling  that  the  active  process  had  been  en- 
tirely stopped  as  suggested  by  the  absence  of  any  posi- 
tive findings  either  in  the  blood  serum  or  in  the  spinal 
fluid.  We  should  perhaps  conclude  that  there  was  no 
more  activity  in  this  case  and  that  the  crises  were  due  to 
the  changes  that  had  already  taken  place  in  the  nerve 
tissue  and  which  could  no  longer  be  changed. 


370 


TREATMENT 


The  literature  is  in  doubt  concerning  (in  fact  is 
preponderantly  against)  the  success  of  treatment 
in  PARETIC  NEUROSYPHILIS  ("general  par- 
esis ").  Our  experience  has  yielded  a  number  of 
apparently  successful  results  through  systematic 
intensive  intravenous  salvarsan  therapy.  Example. 


Case  112.  Albert  Forest  had  always  been  a  successful 
salesman,  but  in  the  middle  of  March,  in  his  46th  year,  he 
was  arrested  for  grabbing  a  purse  from  a  woman  in  front  of 
a  theatre  and  running  down  the  street  with  it.  In  court, 
Forest  acted  strangely  and  he  was  sent  to  the  Psychopathic 
Hospital  for  observation.  Upon  investigation,  it  appeared 
that  his  wife  thought  he  had  been  showing  mental  changes 
for  about  a  year.  For  example,  he  would  embrace  his  wife 
on  a  street  car,  or  refuse  to  pay  her  fare.  He  once  attempted 
to  hit  his  son  on  the  head  with  a  red-hot  poker.  Now  and 
then  he  would  become  sleepy  and  stupid.  He  looked  rather 
older  than  his  age  and  had  a  coarse  tremor  of  the  hands. 
Otherwise,  no  change  could  be  detected  in  the  physical 
examination,  either  neurologically  or  otherwise.  As  for  the 
manual  tremor,  Forest's  wife  gave  a  history  of  considerable 
alcoholic  indulgence  on  his  part. 

For  several  days,  nothing  abnormal  could  be  detected  in 
the  man;  and  in  particular,  his  memory  for  both  remote  and 
recent  events  was  very  good  and  his  knowledge  of  current 
events  was  good.  Simple  arithmetic  was  easy  to  him. 

One  evening  his  temperature  was  found  to  be  104°  F.  and 
no  cause  could  be  discerned  for  this.  The  next  morning, 
Forest  was  discovered  in  a  stupor,  with  a  complete  right 
hemiplegia.  The  Babinski  reflex,  the  Oppenheim  reflex,  and 
ankle  clonus  had  appeared  on  the  right  side,  and  the  right 
arm  was  spastic. 

However,  all  symptoms  of  this  paralysis  had  disappeared  < 
by  four  o'clock  in  the  afternoon,  and  the  paralytic  phenomena  f 
were  replaced  with  violence.  The  patient  fought  with  the 


TREATMENT  371 

attendants  and  for  some  time  remained  extremely  difficult  to 
manage,  being  confused  and  subject  to  outbreaks  of  violence 
with  destruction  of  furniture  and  other  property  about  the 
ward. 

Diagnosis.  At  first  we  were  naturally  inclined  to  dismiss 
the  case  with  a  diagnosis  of  alcoholism.  The  transient 
hemiplegia  at  once  raised  a  considerable  question  of  brain 
syphilis  or  of  brain  tumor. 

The  W.  R.  of  the  serum  was  doubtful.  The  spinal  fluid 
yielded,  besides  marked  excess  of  albumin  and  much  globulin, 
also  a  "  paretic  "  gold  sol  reaction  and  75  cells  per  cmm. 
The  W.  R.  was  positive. 

Treatment.  The  patient  was  given  injections  of  salvar- 
san,  0.6  gram,  twice  a  week,  with  potassium  iodid.  After 
a  few  weeks  improvement  followed,  and  after  several  months 
all  the  laboratory  tests  became  negative,  the  patient  was 
apparently  perfectly  normal  mentally  and  was  discharged 
from  the  hospital,  and  has  remained  well  for  18  months 
without  further  treatment.  The  serum  W.  R.  has  con- 
tinued to  be  negative. 

1.  What  is  the  significance  of  the  so-called  "  doubtful  " 

W.  R.  ?  Where  there  is  not  a  complete  uniformity  the 
results  of  the  strong  and  weak  antigens  (see  appendix 
on  technique  of  Wassermann  reaction)  the  result  is 
reported  as  doubtful.  In  the  majority  of  instances 
repetitions  will  give  a  strong  positive  reaction. 

2.  Is  the  case  of  Forest  to  be  regarded  as  one  of  general 

paresis?  Sometimes  such  cases  are  termed  in  the 
literature  syphilitic  pseudoparesis  (see  case  Burkhardt 
(58)).  The  differential  diagnosis  of  this  group  is 
entirely  therapeutic.  There  are,  unhappily,  no  labora- 
tory tests  which  will  suffice  in  the  present  stage  of 
knowledge  to  differentiate  a  case  of  so-called  pseudo- 
paresis  from  general  paresis.  We  are  inclined  to  term 

the  case  one  of  GENERAL  PARESIS,  with  recovery,  or, 

at  all  events,  with  remission. 


372  TREATMENT 


The  literature  is  in  doubt  concerning  (in  fact  is 
preponderantly  against)  the  success  of  treatment 
in  PARETIC  NEUROSYPHILIS  ("general  par- 
esis ")•  Our  experience  has  yielded  a  number  of 
apparently  successful  results  through  systematic 
intensive  intravenous  salvarsan  therapy.  Example. 


Case  113.  We  present  the  case  of  Gussie  Silverman,  a 
housewife,  35  years  of  age,  among  other  reasons,  for  its  social 
interest.  The  case  is,  on  the  whole,  sufficiently  typical  of 
GENERAL  PARESIS.  Physically,  for  example,  the  pupils  failed 
to  react  to  light  and  accommodation  and  were  unequal,  the 
right  being  larger  than  the  left.  The  knee-jerks  were  sluggish 
though  equal.  The  ankle-jerks  could  not  be  obtained.  The 
abdominal  reflexes  were  not  obtained.  Otherwise,  there  was 
no  reflex  disorder. 

From  the  laboratory  point  of  view,  the  W.  R.  was  positive 
in  the  blood  and  in  the  spinal  fluid.  There  were  80  cells  per 
cmm.  and  there  were  an  appropriate  globulin  and  albumin 
reactions.  Mrs.  Silverman  was  rather  poorly  nourished  and 
had  a  slight  edema  of  the  ankles. 

Mentally,  she  was  found  on  admission  to  be  markedly 
depressed.  It  appeared  that  during  a  recent  pregnancy, 
terminated  by  the  birth  of  a  7-months  child,  she  had  fainted 
several  times  a  day,  that  since  the  confinement  she  had  been 
very  nervous,  that  she  had  been  asking  her  husband  not  to 
send  her  away,  that  she  had  refused  to  leave  the  house,  that 
she  had  become  excited  even  to  the  point  of  injuring  herself, 
especially  at  night,  and  that  she  would  go  so  far  as  to  scratch 
her  husband,  shortly  afterward  being  very  sorry  for  her  per- 
formances. Before  this  last  pregnancy  there  had  been  four 
others  and  the  resulting  children  were  all  apparently  in  good 
health.  Except  for  the  fainting  spells  during  the  pregnancy, 
it  would  not  appear  that  the  story  just  told  is  at  all  charac- 
teristic of  paresis. 


TREATMENT  373 

However,  in  the  hospital  Mrs.  Silverman  could  hardly  be 
got  to  answer  questions,  continually  saying,  "  You  know  what 
it  is;  I  don't  have  to  tell  you."  She  claimed  so  marked  a 
degree  of  confusion  as  not  to  know  where  she  was  and  what 
she  was  doing.  She  would  beg  despondently  that  something 
be  done  for  her,  and  iterate  and  re-iterate  these  claims. 
There  appeared  to  be  a  marked  degree  of  amnesia.  Some 
one,  she  felt,  had  controlled  her  thoughts  and  made  her  do 
things  she  did  not  want  to  do  and  say  things  she  did  not  want 
to  say,  things  she  did  not  know  she  was  about  to  say.  She 
said,  "  I  feel  like  jumping  around.  I  couldn't  believe  myself 
as  if  I  am  me.  Some  one  is  making  me  jump  around.  I  used 
to  hear  him  talking.  I  don't  know  who  it  is.  I  used  to  keep 
my  eyes  open  and  I  couldn't  move.  I  feel  only  I  would  like 
to  talk,  and  talk,  and  talk,  and  talk  all  the  time.  It  seems  to 
me  that  some  one  talks  in  me.  I  couldn't  sleep  for  five  min- 
utes. My  God,  I  wish  I  could  sleep!  I  used  to  feel  something 
in  my  heart.  I  used  to  faint.  It  seems  to  me  I  used  to  see 
a  funny  thing.  What  it  was  I  can't  tell.  It  used  to  talk  to 
me,  make  me  get  out  of  bed,  throw  me  about,  make  me  do 
things.  O,  I  don't  know  what  it  was." 

These  not  entirely  characteristic  mental  symptoms,  to- 
gether with  the  suggestive  physical  signs  and  the  laboratory 
examination,  caused  treatment  to  be  instituted ;  under  which 
treatment  (intravenous  injections  of  salvarsan)  she  improved 
rapidly.  Mental  symptoms  disappeared  under  the  ad- 
ministration of  12  injections  of  salvarsan  within  two  months. 
Moreover,  the  spinal  fluid  became  entirely  negative.  Two 
and  a  half  years  have  now  elapsed  since  her  discharge  and  she 
has  shown  no  return  of  symptoms.  The  serum  W.  R.  has 
always  remained  negative  although  there  has  been  no  treat- 
ment since  leaving  the  hospital.  There  has,  however,  been 
no  change  in  the  reflexes,  which  remain  as  on  admission.  The 
7-months  baby  has  continued  to  be  perfectly  healthy.  Its 
W.  R.  is  negative,  as  are  the  W.  R.'s  of  the  husband  and  the 
other  three  children.  It  must  seem  surprising  that  a  healthy 
child  could  have  been  born  from  a  mother  with  generalized 
syphilis  as  in  this  case.  However,  perhaps  there  are  more 
instances  than  we  imagine  like  the  case  of  baby  Silverman. 


374  TREATMENT 

1.  May  a  patient  be  considered  permanently  cured  although 

there  has  been  no  recurrence  of  symptoms  for  2|  years 
and  although  the  Wassermann  has  remained  negative? 
One  would  hesitate  to  give  a  definite  statement  that  the 
patient  was  cured  until  more  time  had  elapsed.  It  is 
quite  possible  that  spirochetes  may  be  lurking  in  some 
portion  of  the  body  without  causing  the  production  of 
symptoms  or  Wassermann  bodies  and  yet  ready  to 
break  out  at  any  time.  This  hypothesis  has  added 
weight  from  the  recent  work  of  Warthin  already  quoted. 
We  advise  examination  of  this  patient  at  intervals  of 
not  longer  than  six  months  for  a  good  many  years. 

2.  Should  the  course  under  treatment  cause  us  to  change 

the  diagnosis?  It  has  often  been  stated  that  a  differ- 
ential point  between  cerebrospinal  syphilis  and  general 
paresis  is  the  reaction  to  treatment,  that  is,  that  a  case 
which  recovers  could  not  be  general  paresis.  Head 
and  Fearnsides  state  that  if  six  months  after  beginning 
of  treatment  the  spinal  fluid  has  become  negative,  the 
case  should  be  considered  as  one  of  cerebrospinal 
syphilis  and  not  general  paresis.  We  do  not  feel  ready 
to  concur  in  this  view  as  we  know  of  no  similar  logic  in 
medicine.  We  have  many  cases  in  which  a  spinal 
fluid  has  remained  positive  for  six  months  and  later 
become  negative,  so  that  where  the  symptoms  shown 
are  those  of  paretic  neurosyphilis,  we  are  inclined  to  con- 
sider the  case  such  until  such  time  as  more  definite 
evidence  checked  by  post  mortem  examination  causes 
us  to  change  this  point  of  view. 

3.  Do  the  reflexes  change  under  treatment?     The  signs  of 

spasticity  often  do  disappear  under  treatment  and  also 
when  there  is  no  treatment.  A  few  instances  have 
been  reported  in  the  literature  where  Argyll-Robertson 
pupils  are  said  to  have  altered  to  normal.  It  has  never 
been  our  good  fortune  to  see  such  a  change  nor  have 
we  seen  an  absent  knee-jerk  become  normal,  as  has 
also  been  reported,  except  where  it  is  the  result  of 
pyramidal  tract  disease  superimposed  upon  the  pos- 
terior column  sclerosis  causing  a  return  of  reflex.  This, 
of  course,  is  not  to  be  considered  as  a  return  of  the 
normal.  (See  Case  I.) 


TREATMENT  375 


Some  RESULTS  of  systematic  intravenous  salvar- 
san  therapy  are  PARTIAL  (e.g.,  clinical  recovery 
and  persistence  of  positive  laboratory  tests). 


Case  114.  Walter  Henry  was  an  undertaker  in  a  small 
town.  He  was  married  and  the  father  of  two  healthy  children. 
In  May,  1914,  he  began  to  lose  his  appetite.  He  felt  restless 
and  seemed  to  be  losing  his  grip,  and  in  August  he  repaired 
to  a  sanatorium,  where  he  remained  for  two  months.  Shortly 
after  leaving  the  sanatorium,  he  fainted  one  day,  while  dig- 
ging a  grave,  during  a  spell  of  great  heat.  Since  that  time 
there  had  been  numerous  "  weak  spells,"  with  headaches  and 
general  debility,  insomnia,  and  loss  of  weight. 

In  February,  1916,  Mr.  Henry  came  to  the  hospital  for 
advice,  but  the  trip  from  a  distant  part  of  the  state  was  ap- 
parently such  a  strain  for  him  that  shortly  after  admission 
he  collapsed.  There  were  no  convulsive  movements  in  this 
collapse,  but  the  patient  was  confused  and  his  breathing  was 
rapid  and  stertorous.  The  semi-stupor  lasted  for  about  48 
hours.  Upon  recovery  from  the  stupor,  Henry  was  found 
entirely  disoriented,  much  confused,  and  laboring  under  the 
belief  that  he  was  digging  a  grave.  After  a  time  he  again 
fell  into  a  stupor  and  his  temperature  rose  to  103°  F. 

The  emaciation  of  this  man  was  striking  and  unusual, 
but  systematic  physical  examination  showed  no  special  dis- 
ease. Neurologically,  there  were  marked  tremors,  and  there 
were  purposeless  movements  of  the  arms.  There  was  a 
marked  speech  defect.  The  pupils  were  dilated,  regular,  and 
equal,  and  reacted,  though  slightly,  to  light.  Nothing  ab- 
normal was  noted  upon  systematic  examination  of  the  reflexes. 

The  W.  R.  was  strongly  positive  in  the  blood  and  in  the 
spinal  fluid;  the  gold  sol  reaction  was  typically  "paretic"; 
there  were  16  cells  per  cmm.,  globulin  was  present,  and  albumin 
was  greatly  increased. 

The  diagnosis  GENERAL  PARESIS  was  accordingly  made,  and 
treatment  instituted.  Intravenous  injections  of  arsenobenzol, 


376  TREATMENT 

at  first,  and  later  of  diarsenol,  were  given,  as  a  rule  twice  a 
week  (usual  dose,  0.6  of  a  gram).  Mercurial  injections  and 
potassium  iodid  were  also  given.  This  treatment  was  con- 
tinued as  the  patient  began  to  improve.  The  improvement 
was  of  such  a  degree  that  at  the  end  of  four  months,  Mr. 
Henry  returned  to  his  home  and  his  work.  He  had  had  30 
intravenous  injections  of  salvarsan  substitutes.  Despite  the 
treatment  and  the  clinical  improvement,  the  laboratory  tests 
remained  essentially  unchanged.  The  W.  R.'s  of  the  blood 
and  spinal  fluid  remained  strongly  positive,  as  well  as  also  the 
globulin  and  albumin;  the  gold  sol  reaction  was  still  "paretic"; 
the  cells  stood  at  one  per  cmm.  The  patient  has  continued 
antisyphilitic  treatment  since  leaving  the  hospital,  and  has 
remained  apparently  well,  with  good  insight  into  his  condition. 

1.  What  is  the  significance  of  a  temperature  of  103°  in  a 

paretic  without  signs  of  infection  and  a  normal  leuco- 
cyte count?  Temperatures  of  this  type  are  not  infre- 
quent in  the  course  of  general  paresis.  They  are  usually 
spoken  of  as  "  paretic  temperatures."  Their  meaning 
is  not  understood,  but  they  are  often  stated  to  be  due  to 
a  disturbance  of  the  heat-regulating  mechanism.  Such 
temperatures  may  remain  elevated  for  a  considerable 
period  of  time,  but  the  elevation  may  be  very  transi- 
tory. At  times  they  vary,  like  septic  temperatures. 

2.  What  can  be  argued  from  the  fact  that  the  cell  count 

became  normal?  If  thorough  antisyphilitic  treatment 
is  vigorously  given,  it  will  be  found  that  in  the  vast 
majority  of  cases  of  neurosyphilis  the  cell  count  will 
return  to  normal.  It  matters  not  whether  the  treat- 
ment be  intravenous  or  subdural.  It  is  very  difficult, 
however,  to  obtain  this  result  in  general  paresis  by  the 
use  of  mercury  alone.  It  cannot,  however,  be  urged 
that  this  finding  has  any  great  prognostic  significance 
as  it  occurs  in  the  cases  which  do  poorly  as  well  as  in 
those  which  recover  symptomatically. 

3.  Is  it  safe  to  give  large  doses  of  salvarsan  to  a  patient  in 

a  stupor?  It  is  not  a  good  plan  to  give  a  large  dose  to 
such  a  patient  on  account  of  the  danger  of  sudden 
death.  This  is  probably  due  as  much  to  the  strain 
put  on  the  heart  as  it  is  to  any  effect  on  the  nervous 
system,  or  specific  arsenic  effect.  In  this  particular 
instance,  a  dose  of  0.15  gm.  was  the  initial  injection 
and  this  was  increased  five  centigrams  per  injection. 


TREATMENT  377 


IMPROVEMENT  IN  PARETIC  NEUROSYPH- 
ILIS  ("general  paresis")  may  become  evident 
only  after  several  months  of  intensive  treatment. 


Case  115.  Henry  Ryan  was  a  shipping  clerk,  54  years  of 
age,  who  was  brought  to  the  hospital  following  a  convul- 
sion. For  a  few  months  preceding  this  period,  Mr.  Ryan 
had  been  failing  in  his  abilities.  He  had  been  very  forgetful, 
showed  no  energy,  and  had  become  very  irritable.  He  also 
complained  of  insomnia  and  of  feeling  nervous. 

On  admission  to  the  hospital,  the  most  striking  feature  in 
the  mental  situation  was  that  he  claimed  that  he  had  not 
slept  a  wink  for  three  months,  and  each  day  he  would  solemnly 
affirm  that  he  had  not  slept  at  all  the  preceding  night,  although 
the  records  might  show  that  he  had  slept  eight  hours.  Argu- 
ment was  of  no  avail  against  this  conviction.  In  addition, 
his  memory  was  very  poor;  he  showed  little  knowledge  of 
current  events,  and  had  no  ability  with  arithmetical  problems. 

Neurologically  viewed,  the  points  of  chief  significance  were 
contracted  immobile  pupils  and  a  speech  defect,  especially 
noticeable  on  the  repetition  of  test  phrases.  The  whole 
picture  was  suggestive  of  general  paresis,  and  this  diagnosis 
was  confirmed  by  the  laboratory  findings.  It  was  found  that 
the  W.  R.  was  positive  in  the  blood  and  spinal  fluid,  that 
there  was  a  pleocytosis,  positive  globulin  reaction,  excess  of 
albumin,  and  a  "  paretic  "  gold  sol  reaction.  Consequently, 
the  diagnosis  of  GENERAL  PARESIS  seemed  justified,  although 
the  patient  denied  any  knowledge  of  a  syphilitic  infection. 

Treatment  in  this  case  consisted  of  intravenous  injections 
of  salvarsan,  diarsenol,  or  arsenobenzol,  whichever  drug  was 
most  easily  obtainable,  given  twice  a  week  in  doses  of ^0.6 
gram  each.  In  addition,  he  was  given  occasional  injections 
of  mercury  salicylate  as  well  as  potassium  iodid  by  mouth. 
Once  or  twice  a  week,  40  to  60  cc.  of  spinal  fluid  were  with- 
drawn. Under  this  treatment  for  a  period  of  three  months, 
the  patient  showed  no  improvement  whatsoever,  either  in 


378  TREATMENT 

his  mental  condition  or  in  the  laboratory  findings.  However, 
treatment  was  faithfully  persevered  in,  and  shortly  after  the 
three  months,  improvement  began  to  be  noticed.  At  first, 
the  patient  began  to  admit  that  possibly  he  may  have  slept 
a  few  winks  some  time  during  the  previous  six  months,  for 
he  said  he  realized  it  was  not  possible  for  a  man  to  live  without 
sleep  for  that  period.  Then  he  began  to  admit  that  he  might 
have  slept  a  few  hours  during  the  night,  and  later  that  he 
was  sleeping  pretty  fairly.  His  memory  also  showed  im- 
provement. His  general  attitude  showed  alertness,  and  he 
began  to  interest  himself  in  his  surroundings  and  in  the  events 
of  the  world,  and  finally  he  gained  complete  insight  into  his 
condition. 

In  the  meantime,  that  is  after  three  months  of  treatment, 
the  laboratory  findings  began  to  grow  weaker.  The  gold  sol 
reaction  was  the  first  to  decrease  in  strength,  and  after  four 
months  of  treatment,  it  vacillated  between  negative  and  a 
mildly  positive  "  syphilitic  "  reaction.  Then  the  globulin 
and  albumin  became  less  in  amount,  and  the  W.  R.  began 
dropping  off  in  the  o.i  and  0.3  cc.  dilutions.  As  is  usually 
true  in  those  cases  of  neurosyphilis  that  receive  adequate 
treatment,  the  cell  count  early  dropped  to  normal.  The 
W.  R.  in  the  blood  serum,  however,  remained  positive. 

As  the  patient's  condition  seemed  so  much  better,  he  was 
allowed  to  leave  the  hospital  at  the  end  of  five  months.  He 
took  things  easily  for  the  following  seven  months,  and  then, 
after  being  out  of  employment  for  the  period  of  a  year,  as  his 
health  continued  good,  he  decided  to  return  to  work.  Before 
doing  so,  he  entered  the  hospital  again  for  a  lumbar  puncture. 
At  this  time,  it  was  found  that  the  cell  count  was  normal, 
there  was  a  very  faint  trace  of  globulin,  possibly  a  slight  in- 
crease above  normal  albumin  content,  and  a  very  mild  gold 
reaction.  The  W.  R.  in  the  spinal  fluid  was  negative  includ- 
ing the  i.o  cc.  dilution;  the  blood  serum  remained  positive. 

The  patient  then  returned  to  his  old  position  and  has  done 
satisfactorily  for  the  past  six  months.  During  this  entire 
time,  he  has  been  coming  to  the  hospital  for  treatment: 
during  the  major  portion  of  the  time,  about  once  in  two  weeks ; 
of  late,  once  in  four  weeks. 


TREATMENT  379 

The  significant  point  in  this  case  is  that  improvement  did 
not  show  itself  until  after  more  than  three  months  of  intensive 
treatment,  and  then  the  improvement  was  synchronous  with 
a  weakening  of  the  spinal  fluid  tests. 

It  is  further  significant  that  his  mental  and  physical  condi- 
tion was  good  before  the  tests  had  reached  anything  like 
normal;  and  that  under  treatment,  these  tests  continued 
to  grow  weaker  and  weaker,  until  at  the  end  of  a  year,  they 
were  practically  negative. 

The  case  further  illustrates  the  enormous  number  of  in- 
jections of  salvarsan  preparations  that  may  be  given  to  a 
patient  without  causing  any  appreciable  damage  to  the  gen- 
eral health  or  to  the  kidney  function.  Mr.  Ryan  has  had 
more  than  60  injections. 

1 .  How  soon  after  treatment  is  instituted  does  improvement 

usually  occur  in  paretic  neurosyphilis?  In  our  ex- 
perience improvement  usually  shows  itself  in  from  two 
or  three  months  of  treatment.  Occasionally  the  im- 
provement may  be  very  marked  shortly  after  treatment 
is  commenced,  that  is,  after  three  or  four  injections  of 
salvarsan.  This  is  not,  however,  the  rule  and  as  in 
the  case  of  Ryan,  it  may  be  only  after  more  than  three 
months  that  improvement  is  seen.  This  means  that 
in  the  treatment  of  these  cases  patience  must  be  exer- 
cised and  much  work  done. 

2.  What  is  the  point  of  withdrawing  large  amounts  of  spinal 

fluid  as  in  the  case  of  Henry  Ryan?  It  has  been  stated 
that  the  withdrawal  of  40  or  more  cc.,  of  spinal  fluid 
while  the  patient  is  under  treatment  has  the  effect  of 
reducing  the  intraspinous  and  intracranial  pressure  and 
thereby  allowing  the  drug  to  diffuse  into  the  nervous 
tissue  better  than  it  would  do  under  ordinary  condi- 
tions. How  much  truth  there  is  in  this  contention  it  is 
difficult  to  say  and  there  is  as  yet  no  experimental  evi- 
dence to  confirm  this  contention.  As  a  matter  of  fact, 
the  spinal  fluid  in  cases  of  paresis  is  usually  under 
increased  pressure  and  it  is  at  least  plausible  to  conceive 
that  a  reduction  of  this  pressure  may  give  some  symp- 
tomatic relief. 


380  TREATMENT 


Evidence  of  the  activity  of  syphilis  outside  the 
central  nervous  system  may  be  seen  in  cases  of 
neurosyphilis  despite  intensive  treatment. 


Case  116.  William  Rossetti  was  a  speculator,  43  years  of 
age,  when  he  was  brought  to  the  Psychopathic  Hospital  on 
account  of  an^outbreak  in  which  he  smashed  a  showcase  at 
the  store  where  his  sweetheart  was  employed;  he  caused  so 
much  commotion  that  he  was  arrested. 

On  admission,  he  was  very  excited,  talking  loudly  and  at 
length.  For  some  days  it  was  very  difficult  to  manage  him, 
he  was  so  active.  At  any  moment,  he  would  insist  upon 
undressing  and  taking  physical  culture  exercises.  He  was 
very  euphoric  and  expansive,  and  had  no  insight  into  his 
condition. 

Physically,  he  was  a  powerfully-built  man  and  in  very  good 
physical  condition  except  for  an  iritis  and  moderate  thickening 
of  the  peripheral  arteries.  The  neurological  signs  of  import- 
ance were  Argyll-Robertson  pupils,  and  absent  knee-jerks 
and  ankle- jerks.  With  these  findings  in  mind,  a  tentative 
diagnosis  of  GENERAL  PARESIS  was  made,  and  this  was  sub- 
stantiated by  the  laboratory  tests,  which  gave  positive  W. 
R.'s  in  blood  and  spinal  fluid,  globulin,  excessive  albumin, 
slight  pleocytosis,  and  a  "  paretic  "  gold  sol  reaction. 

When  the  patient's  mental  condition  was  somewhat  better, 
he  gave  a  history  of  syphilitic  infection  15  years  before,  for 
which  he  had  had  almost  continuous  treatment.  As  a  matter 
of  fact,  treatment  had  been  pretty  strenuous  because  he  had 
recurring  skin  lesions  and  iritis.  It  was  practically  impos- 
sible to  get  the  skin  lesions  to  heal  with  mercury,  and  it  was 
not  until  salvarsan  was  introduced  that  a  good  result  was 
obtained  in  this  respect.  After  one  or  two  injections  of  this 
drug,  the  skin  lesion  disappeared  and  has  never  returned. 
However,  at  least  once  a  year,  he  has  had  attacks  of  iritis, 
and  for  this  reason  was  still  being  treated  for  syphilis  at  the 
outbreak  of  his  psychosis. 


TREATMENT  381 

He  was  at  once  placed  on  more  strenuous  antisyphilitic 
treatment  in  the  form  of  diarsenol,  semi-weekly,  aided  by 
mercury  injections.  After  a  few  months  of  this  treatment, 
his  mental  condition  improved  so  much  that  he  seemed  to  be 
entirely  normal.  Treatment  was  continued,  however,  with- 
out any  abatement,  and  it  was  of  great  interest  to  note  at  the 
end  of  five  months  of  such  treatment  that,  although  mentally 
he  seemed  entirely  well,  he  had  an  attack  of  iritis,  which  was 
considered  as  a  sign  of  active  syphilis.  This  would  appear 
to  indicate  the  great  difficulty  of  getting  results  in  certain 
cases  of  syphilis  with  any  drugs  at  our  command  at  present, 
as  in  the  iritis  we  are  dealing  with  a  condition  which  as  a 
rule  reacts  fairly  readily  to  antisyphilitic  remedies. 

1.  Are  there  different  strains  of  spirochetes  showing  various 

degrees  of  malignancy?  This  question  has  been  dis- 
cussed at  length  in  the  literature  but  there  is  no  satis- 
factory answer  at  the  present  time.  We  must  always 
consider  the  reaction  of  the  organism  and  the  host ;  and 
it  is  true  in  syphilis,  as  in  every  other  disease,  that  in 
some  individuals  it  is  more  difficult  to  get  any  therapeu- 
tic results  than  in  others. 

2.  Was  the  failure  to  obtain  results  by  long  years  of  treat- 

ment due  to  "drug  fastness"  of  the  spirochetes?  It  has 
been  held  that  the  organism  of  syphilis  will  develop  an 
immunity  after  a  time  to  mercury  and  arsenic  prepara- 
tions. This  led  Fournier  to  recommend  intermittent 
treatment  as  more  efficient  than  continuous  treatment. 
Noguchi  has  shown  that  in  test-tube  experiments,  the 
spirochetes  develop  a  tolerance  to  increasing  doses  of 
arsenic.  It  must  be  emphasized,  however,  that  this  find- 
ing has  not  been  established  for  the  conditions  in  vivo. 
Another  explanation  of  the  failure  of  treatment  in  certain 
instances  has  been  offered  by  McDonagh,  who  describes 
a  life  cycle  of  the  organism  of  syphilis  under  the  name  of 
cytorrhyctes  luis,  of  which  he  believes  the  spirochete  to  be 
merely  one  form,  the  other  forms  not  being  affected  by 
arsenic  or  mercury. 


382  TREATMENT 


Some  results  of  systematic  intravenous  salvarsan 
therapy  in  PARETIC  NEUROSYPHILIS  ("  gen- 
eral paresis  ")  are  partial  in  the  sense  that  with 
clinical  recovery  the  laboratory  tests  remain  par- 
tially or  less  strongly  positive. 


Case  117.  Annie  Martin  was  a  charwoman,  37  years  of 
age.  She  had  applied  for  relief  at  a  general  hospital,  to  which 
she  was  admitted  on  the  suspicion  of  nephritis;  but  upon 
admission  she  became  markedly  excited  and  noisy,  and  spoke 
of  seeing  angels  and  hearing  God  speak  to  her.  As  the  at- 
tendants were  unable  to  quiet  her,  she  was  promptly  trans- 
ferred to  the  Psychopathic  Hospital.  She  maintained  that 
she  had  been  sent  to  the  Psychopathic  Hospital  through  the 
spite  of  the  general  hospital  doctors,  and  she  claimed  that 
other  people  were  also  attempting  to  work  her  harm  for  the 
purpose  of  taking  her  children  from  her.  Visual  and  auditory 
hallucinations  were  marked,  as  was  the  patient's  loquacity, 
irritability,  and  flight  of  ideas.  However,  she  seemed  entirely 
oriented  and  her  memory  appeared  to  be  intact.  She  was 
able  to  explain  somewhat  clearly  her  supposed  condition. 
The  voices  told  her  that  somebody  was  after  her  and  that 
her  soul  belonged  to  the  devil;  that  she  was  to  be  married 
but  that  her  soul  was  to  be  damned.  These  voices  probably 
belonged  to  priests.  She  was  under  the  impression  that  she 
was  going  to  be  sent  to  an  electric  chair  and  said,  "  I  think  I 
am  coming  to  the  end  and  I  want  a  pair  of  rosary  beads 
before  the  end  comes." 

This  patient's  pupils  were  markedly  unequal  and  entirely 
stiff  to  light  and  accommodation.  Neurologically,  however, 
there  were  no  other  symptoms.  There  was  a  slight  trace  of 
albumin  in  the  urine  and  there  were  no  casts. 

The  psychiatric  diagnosis  in  this  case  would  off-hand 
undoubtedly  be  dementia  praecox.  Yet  the  stiff  pupils  are 
almost  proof  positive  of  neurosyphilis.  If  further  proof 
were  necessary,  it  is  found  in  the  laboratory  tests,  which 


TREATMENT  383 

showed  a  positive  W.  R.  of  the  serum  and  fluid,  with  a 
"  paretic  "  gold  sol  reaction;  there  were  22  cells  per  cmm., 
there  was  excess  albumin,  and  a  positive  globulin  reaction. 
Under  intensive  antisyphilitic  treatment,  there  was  a  slow 
improvement.  After  several  months,  the  patient  was  en- 
tirely free  from  mental  symptoms;  the  spinal  fluid  tests 
became  entirely  negative  except  that  the  gold  sol  reaction 
has  remained  strongly  positive. 

1.  Should   treatment  be  continued  in  the  case  of  Annie 

Martin  in  spite  of  the  clinical  recovery  and  the  negative 
tests  except  the  gold  sol?  We  would  again  emphasize 
that  it  is  unreasonable  to  suppose  that  a  long  standing 
case  of  syphilis  can  be  cured  in  a  period  of  a  few  months 
of  treatment  and  while  the  tests  may  become  negative, 
it  would  seem  foolhardy  to  stop  treatment  on  this 
account.  We  do  know  that  in  many  cases  a  Wasser- 
mann  reaction  remaining  negative  for  many  months 
may  again  become  positive,  indicating  that  the  nega- 
tive reaction  did  not  mean  cure  but  rather  the  absence 
of  the  Wassermann  bodies  in  the  circulation  at  the  time 
the  test  was  made. 

2.  What  is  the  significance  of  the  paretic  gold  sol  reaction 

when  the  other  tests  have  become  negative?  As 
previously  stated,  the  gold  reducing  substance  in  the 
spinal  fluid  seems  to  be  different  from  the  substances 
which  give  the  other  pathological  reactions.  We 
should  feel  in  this  case  that  the  process  which  was 
producing  these  gold  reducing  bodies  had  not  been 
stopped,  in  other  words,  cure  was  not  complete. 

3.  Should  one  make  a  diagnosis  on  the  "  paretic  "  gold  sol 

reaction  alone?  The  so-called  paretic  gold  sol  curve 
is  not  always  indicative  of  general  paresis  or  even  of 
syphilis  but  may  occur  in  non-syphilitic  conditions  as 
brain  tumor,  multiple  sclerosis,  etc.  In  our  experience 
we  have  seen  no  case  of  untreated  neurosyphilis  in 
which  the  gold  sol  alone  was  positive,  that  is,  in  cases 
in  which  therapy  has  not  changed  the  findings  in  the 
spinal  fluid.  In  our  experience  the  gold  sol  reaction 
has  been  fortified  by  one  or  several  of  the  other  tests 
as  the  W,  R,,  globulin  test,  pleocytosis. 


384  TREATMENT 


Some  effects  of  systematic  intravenous  salvarsan 
therapy  in  PARETIC  NEUROSYPHILIS  ("  general 
paresis ")  are  limited  to  the  laboratory  findings 
without  clinical  improvement. 


Two  examples  of  such  limitation  are  offered:  William 
Roberts  (118)  and  John  Silver  (119). 

Case  118.  A  bank  teller,  William  Roberts,  39,  was  sent 
to  the  Psychopathic  Hospital  for  a  depression  so  marked  that 
he  had  become  entirely  unable  to  work  or  care  for  himself. 
The  story  was  that  some  money  had  been  left  him  by  his 
uncle,  that  Roberts  could  not  prove  his  right  to  the  money, 
and  that  depression,  insomnia,  and  occasional  periods  of 
confusion  had  followed  during  a  period  of  about  five  months. 

On  admission,  Roberts  appeared  wholly  disoriented  and 
unable  even  to  give  his  correct  age.  Attention  could  not 
be  held,  and  the  patient  would  slide  off  into  statements  like: 
"  Oh,  I  made  a  mistake,  I  fooled  a  lot  of  people,  I  have  a 
terrible  disease,  they  are  going  to  get  it,  they  are  going  to  get 
me,"  etc.,  etc.  There  was  great  difficulty  in  thinking,  and  a 
marked  reaction  of  fear.  This  cluster  of  phenomena  certainly 
suggested  very  strongly  the  diagnosis  of  manic-depressive 
psychosis. 

Neurologically,  Roberts  proved  quite  negative  except  that 
the  tendon  reflexes  were  very  active  and  the  pupils  reacted 
somewhat  sluggishly  to  light.  The  blood  serum  W._R.  was 
negative.  No  history  of  syphilis  could  be  obtained;  never- 
theless, Roberts  kept  dropping  remarks  about  the  terrible 
disease  from  which  he  was  suffering.  It  seemed  best  to  pro- 
ceed to  lumbar  puncture,  and  the  spinal  fluid  disclosed  a 
positive  W.  R.,  globulin,  increased  albumin,  pleocytosis,  and 
"  paretic  "  gold  sol  reaction. 

The  diagnosis  of  GENERAL  PARESIS  was  accordingly  made. 
During  the  next  year  and  a  half,  no  improvement  was  made; 
a  slight  speech  defect  was  developed,  and  tremors  of  the  hand 
and  tongue  appeared. 


TREATMENT  385 

The  effect  of  treatment  is  particularly  instructive.  Only 
after  18  months  in  the  hospital  was  intensive  antisyphilitic 
treatment  instituted;  but  after  a  few  months  of  this  treat- 
ment the  W.  R.  of  the  spinal  fluid  had  become  negative,  the 
cells  normal  in  number,  globulin  absent,  albumin  present 
only  in  normal  amount.  Only  the  gold  sol  reaction  re- 
mained positive.  It  is  still  of  a  paretic  type.  Treatment, 
however,  did  not  succeed  in  altering  the  patient's  mental 
condition  in  the  slightest.  At  the  end  of  many  months  of 
treatment,  we  still  confront  a  man  showing  marked  psychic 
symptoms  and  a  "  paretic  "  gold  sol  reaction  without  other 
laboratory  signs. 

1.  What  is  the  significance  of  the  practically  negative  tests 

in  this  case  without  clinical  improvement?  One  must 
believe  that  the  tests  became  negative  as  the  result  of 
treatment,  and  that  this  change  in  the  tests  was  due  to 
the  clearing  up  of  some  inflammatory  reactions  which 
were  present.  This  may  mean  that  the  syphilis  had 
been  reduced  to  inactivity  or  latency  if  not  cured,  or 
at  least  that  there  was  no  activity  sufficient  to  cause 
a  positive  W.  R.  in  the  blood  serum,  whereas  whatever 
activity  was  present  in  the  brain  was  in  such  a  region 
that  it  did  not  cause  any  reacting  substances  to  be  cast 
into  the  spinal  fluid.  This  would  not  mean  that  there 
would  necessarily  be  any  return  of  function  already  lost, 
because  this  may  be  considered  as  a  permanent  loss  which 
cannot  be  compensated  for.  As  to  these  tests,  we  now 
feel  that  the  case  should  remain  stationary;  that  is, 
that  no  new  symptoms  will  be  added.  However,  we 
believe  that  it  is  somewhat  premature  with  our  present 
knowledge  to  make  this  claim  very  forcibly,  and  would 
rather  suggest  that  this  case  be  considered  as  demon- 
strating an  interesting  fact,  the  meaning  of  which  can 
be  learned  only  after  a  period  of  years. 

2.  Why  does  the  gold  sol  reaction  remain  strongly  positive 

when  all  the  other  tests  become  negative?  As  already 
pointed  out,  above  (Case  Martin  (117))  there  is  no  known 
rule  about  the  disappearance  of  one  or  other  of  the  ab- 
normal findings  in  spinal  fluid  under  treatment,  and  we 
can  at  present  offer  no  explanation  of  this  phenomenon. 
It  does,  however,  illustrate  how  careful  we  must  be  in 
drawing  any  conclusions  from  tests  in  cases  that  are 
being  treated. 


TREATMENT 


Diminution  in  the  spinal  fluid  tests  may  occur 
in  treated  cases  of  neurosyphilis  without  clinical 
improvement. 


Case  119.  John  Silver,  a  man  29  years  of  age,  presented 
classical  symptoms  of  GENERAL  PARESIS:  He  had  a  convul- 
sion shortly  before  his  admission  to  the  Psychopathic  Hospital, 
his  memory  was  poor,  he  was  only  partially  oriented,  he  was 
very  euphoric  and  expansive  —  thought  he  had  millions, 
that  he  was  the  Czar  of  Russia,  and  so  on.  His  tendon  re- 
flexes were  very  much  increased  and  there  was  a  marked 
speech  defect.  The  W.  R.  of  both  blood  and  spinal  fluid 
were  strongly  positive;  the  spinal  fluid  showed  globulin, 
increased  albumin,  pleocytosis,  and  a  "  paretic  "  gold  sol 
reaction.  There  was,  therefore,  no  question  about  the 
diagnosis,  and  the  patient  was  at  once  put  under  antisyphi- 
litic  treatment.  This  was  continued  for  five  months ;  slowly 
the  intensity  of  the  reactions  in  the  spinal  fluid  diminished. 
At  the  end  of  the  five  months,  there  was  the  very  slightest 
possible  trace  of  globulin,  with  a  doubtful  increase  in  albumin, 
one  cell  per  cmm.,  and  a  mild  syphilitic  gold  sol  reaction. 
The  W.  R.'s  in  the  blood  and  spinal  fluid,  however,  remained 
strongly  positive.  There  was  no  mental  improvement  co- 
incident with  the  weakening  of  the  spinal  fluid  tests,  and  at 
the  end  of  the  five  months,  the  patient  had  a  series  of  con- 
vulsions in  which  he  died. 

This  case  is  given  as  a  contrast  to  Case  Henry  (114)  in  which 
clinical  improvement  occurred  without  diminution  in  labora- 
tory tests;  in  the  case  of  John  Silver,  marked  diminution 
in  the  intensity  of  these  tests  had  no  prognostic  signifi- 
cance. This  was  in  keeping  with  the  condition  as  shown  in 
Case  Roberts  (118)  where,  while  the  gold  sol  was  the  only  test 
to  remain  positive,  the  patient  did  not  improve  mentally. 

I.  What  is  the  explanation  of  the  lessening  of  the  patho- 
logical elements  in  the  spinal  fluid  under  treatment? 
We  have  seen  that  the  various  findings  may  occur  in- 


TREATMENT  387 

dependently  of  one  another,  and  we  must  admit  that 
we  do  not  know  definitely  what  it  signifies,  or  why  one 
may  be  present  or  absent.  It  has  been  held  by  Head 
and  Fearnsides  that  the  findings  in  the  spinal  fluid 
represent  conditions  in  the  spinal  cord  and  spinal 
meninges,  or  at  the  base  of  the  brain  only,  and  not 
conditions  elsewhere.  This  is  in  keeping  with  our 
finding  that  the  gold  sol  reaction  in  the  spinal  fluid 
post  mortem  very  often  differs  from  that  in  the  ven- 
tricular fluids  or  cerebral,  subdural,  and  subpial  fluids. 
And  further,  we  have  found  that  during  life  the  findings 
in  paresis  in  the  spinal  fluid  may  differ  markedly  from 
those  in  the  third  ventricle,  and  that  the  change  in  the 
fluid  in  these  two  areas  under  treatment  may  not  occur 
simultaneously. 


388  TREATMENT 


Systematic  intensive  treatment  of  PARETIC 
NEUROSYPHILIS  ("general  paresis"),  including 
intraventricular  injections  of  salvarsan,  may  en- 
tirely fail. 


Case  120.  James  McGinnis,  aged  39,  came  to  the  hospital 
on  a  stretcher,  semi-conscious,  moaning,  unable  to  reply  to 
questions;  there  were  signs  of  a  right  hemiplegia. 

The  next  day,  McGinnis  cleared  a  little  and  became  able 
to  utter  a  few  words.  His  wife  said  that  he  had  been  en- 
tirely well  up  to  four  years  ago.  At  that  time  he  was  struck 
in  the  eye  by  the  head  of  a  hammer  that  flew  off  the  handle. 
Diplopia  had  developed,  but  disappeared. 

Only  two  years  later  did  a  marked  change  appear.  McGin- 
nis became  careless  as  to  personal  appearance.  Seemed 
absent-minded,  apathetic  and  drowsy;  he  would  fall  asleep 
in  his  chair  or  while  at  work.  He  lost  his  position  and  be- 
came apprehensive,  making  not  very  strenuous  efforts  to 
find  work,  and  finally  consulted  a  physician.  The  physician 
told  him  that  he  had  a  sluggish  liver  and  gave  him  calomel. 

Six  months  later,  McGinnis  was  restored  to  his  position 
as  foreman,  and  his  work  remained  satisfactory  for  some  six 
months.  Then  (about  six  months  before  coming  to  hospital) , 
his  speech  became  slow  and  somewhat  unintelligible.  He 
quit  work,  saying  that  his  speech  was  going  from  him  and 
that  he  might  be  considered  to  be  drunk.  His  memory 
grew  rapidly  worse.  There  was  improvement  after  a  vacation 
and  he  returned  to  work,  but  continued  to  be  ataxic,  com- 
plained of  vertigo,  and  fell  down  several  times,  though 
without  loss  of  consciousness.  On  the  very  day  of  his  ad- 
mission to  the  hospital,  in  attempting  to  get  out  of  bed,  he 
fell,  and  psychotic  symptoms  at  once  appeared.  There 
was  slight  improvement  again  with  entire  disappearance  of 
all  paralysis  after  a  few  days,  a  slow  clearing  up  of  the  speech 
disturbance,  and  a  certain  return  of  memory. 

Physically,  there  was  little  to  note.     Neurologically,  the  left 


TREATMENT  389 

pupil  failed  to  react  to  light.  The  tendon  reflexes  were  all 
very  active,  and  more  active  on  the  left  side.  Other  abnor- 
mal reflexes  were  absent.  Improvement  continued  for  a  num- 
ber of  weeks,  but  the  patient  never  recovered  from  his  speech 
defect,  and  his  memory  remained  impaired.  Irritable  at 
times,  McGinnis  was  for  the  most  part  very  happy  and  sure 
he  would  get  well.  The  W.  R.  of  the  blood  serum  was 
negative,  but  the  spinal  fluid  reaction  was  strongly  positive, 
even  down  to  o.i  cc.  The  globulin  and  albumin  amounts 
were  excessive.  There  was  a  "paretic"  gold  sol  reaction. 
There  were  7  cells  per  cmm.  The  diagnosis  of  GENERAL 
PARESIS  was  made. 

Intravenous  injections  of  salvarsan,  arsenobenzol  or  diar- 
senol  were  made,  and  intramuscular  injections  of  mercury, 
and  potassium  iodid  by  mouth  were  given.  No  real  im- 
provement occurred  after  a  certain  initial  betterment;  the 
spinal  fluid  yielded  no  changes.  Diarsenolized  serum  ac- 
cording to  the  Swift-Ellis  technique  was  then  injected  into 
the  third  ventricle.  Under  this  treatment  also  there  was  no 
change  for  the  better  over  a  period  of  several  months.  The 
patient  died  suddenly  after  a  series  of  convulsions,  apparently 
from  paralysis  of  respiration. 

I.  What  are  the  causes  of  hemiplegia  and  confusion  or 
unconsciousness?  We  must  consider  epilepsy,  brain 
tumor,  cerebral  thrombosis,  cerebral  hemorrhage,  mul- 
tiple sclerosis,  cerebral  spinal  syphilis,  and  general 
paresis. 


390 


TREATMENT 


MILD  TREATMENT,  often  thought  "adequate," 
MAY  FAIL,  WHEN  INTENSIVE  TREATMENT 
PROVES  SUCCESSFUL. 


Case  121.  Arthur  Bright,  a  printer,  had  acquired  syphilis 
in  his  49th  year,  some  six  months  before  examination.  He 
had  been  treated  during  these  six  months  by  three  injections 
of  salvarsan,  injections  of  mercury,  and  mercury  by  mouth. 
He  had  been  apparently  cured  until  about  a  month  before 
admission.  He  had  fallen  without  warning  from  his  chair 
in  a  convulsion  accompanied  by  unconsciousness,  which  lasted 
about  two  hours.  The  patient  had  since  been  feeling  rather 
peculiar.  For  instance,  time  seemed  to  flow  too  rapidly. 
Sometimes  the  patient  had  had  difficulty  in  talking. 

Physically,  nothing  abnormal  could  be  found  either  in 
general  condition  or  neurologically.  The  patient  was,  how- 
ever, incontinent.  Mentally,  he  was  apathetic  and  unalert, 
even  paying  no  attention  to  his  outside  physician  when  he 
came  to  visit  him. 

The  diagnosis  of  cerebrospinal  syphilis  already  suggested 
by  his  history  was  confirmed  by  the  laboratory  tests,  which 
showed  a  positive  serum  and  spinal  fluid  W.  R.,  paretic 
gold  sol  reaction,  41  cells  per  cmm.,  an  excess  of  albumin, 
and  a  positive  globulin  test. 

1.  What  is  the  prognosis  in  cerebrospinal  syphilis  in  the 

early  secondary  stage?  The  prognosis  appears  very 
good  provided  that  intensive  treatment  be  given  and 
provided  that  no  vascular  insult  or  other  focal. destruc- 
tive lesion  occurs  before  treatment  has  had  time  to  do 
its  work. 

2.  Why  did  not  the  "  effective  "  (?)  treatment  for  the  syphilis, 

dating  from  the  primary  lesion,  succeed  in  staving  off 
the  cerebrospinal  syphilis?  It  remains  a  question 
whether  the  treatment  by  three  injections  of  salvarsan 
was  efficient  in  this  particular  case.  Of  course,  it  may 
prove  true  that  no  treatment  whatever  in  the  present 
stage  of  knowledge  will  stave  off  cerebrospinal  symp- 
toms in  certain  cases. 


TREATMENT  39! 

Treatment:  Bright  was  given  intravenous  injections  of 
diarsenol  twice  a  week,  with  occasional  injections  of  mercury 
salicylate.  After  two  weeks,  the  patient  seemed  markedly 
improved,  and  continued  to  improve  rapidly.  He  was  symp- 
tomatically  well  at  six  weeks.  The  spinal  fluid  had  then 
become  negative,  although  the  serum  W.  R.  had  remained 
positive. 

After  discharge  from  the  hospital,  Bright  returned  to  his 
work,  but  continued  to  take  the  diarsenol  treatment  weekly, 
and  two  months  later  the  serum  W.  R.  became  negative. 

Small  injections  of  diarsenol  at  intervals  of  a  month  were 
continued,  and  Bright  remained  perfectly  well  for  four 
months,  when  a  peculiar  seizure  developed  and  lasted  for 
several  hours.  This  seizure  consisted  in  a  sort  of  somnam- 
bulism in  which  Bright  stood  up  at  a  table,  making  marks 
on  paper,  and  could  not  be  persuaded  to  desist.  After  this 
seizure,  Bright  re-entered  the  hospital,  again  showed  no 
mental  or  physical  symptoms  and  no  abnormalities  of  blood 
or  spinal  fluid. 

3.  What  is  the  explanation  of  this  seizure?  It  is  possibly 
due  to  a  small  vascular  insult,  for  which  potassium 
iodid  may  be  suggested  with  precautions  as  to  hygiene 
and  continued  observation.  He  has  since  remained 
entirely  well. 


392 


TREATMENT 


Another  example  where  MILD  MEASURES 
(though  conceived  to  be  "adequate")  SEEMED 
TO  BE  LEADING  TO  FAILURE;  INTENSIVE 
THERAPY  SUCCESSFUL. 


Case  122.  Levi  Morovitz,  a  waiter,  39  years  of  age,  came 
to  the  hospital  with  evidences  of  an  old  left  hemiplegia,  in- 
cluding the  left  side  of  the  face  (there  was  a  left-sided  Bab- 
inski,  Gordon,  and  Oppenheim,  and  all  the  reflexes  were 
fairly  active;  sluggish  pupil  reactions,  Rombergism,  and 
speech  defect).  Morovitz  was  much  depressed,  very  slow 
in  thinking  processes,  had  a  marked  memory  disturbance  in 
general  and  apparently  much  deterioration  mentally. 

A  history  was  obtained  to  the  effect  that  Morovitz  had 
acquired  syphilis  at  about  33,  but  that  he  had  received 
practically  continuous  treatment  ever  since  at  a  dispensary. 
He  had,  in  fact,  received  four  injections  of  salvarsan  a  year 
before  coming  to  the  hospital.  Of  late,  Morovitz  had  become 
much  more  cheerful  and  talkative,  imagining  he  could  do 
great  things  if  he  had  money.  He  had  begun  to  eat  very 
rapidly  and  to  be  very  nervous.  His  feet  had  begun  to  drag; 
a  distinct  speech  defect  developed,  but  from  this  he  had  re- 
covered. About  six  weeks  before  entrance,  Morovitz  had  a 
shock,  which  left  him  with  the  left  hemiplegia  above  men- 
tioned and  with  considerable  headache. 

Even  while  the  preliminary  examination  was  being  per- 
formed, Morovitz  developed  a  minor  seizure  without  loss  of 
consciousness.  First  came  severe  pain  over  the  frontal 
region,  which  grew  in  severity  so  that  the  patient  held  his 
head  in  his  hands.  A  bit  later,  twitching  movements  began 
in  the  thumb  and  in  the  fingers  of  the  left  hand,  and  the 
small  muscles  of  the  extensor  group  of  the  thumb  and  third 
finger  showed  contractions.  These  contractions  grew  more 
general  and  the  excursions  of  the  fingers  greater,  until  finally 
every  finger  of  the  left  hand  became  involved,  whereupon 
movements  of  the  same  sort,  though  of  smaller  amplitude, 


TREATMENT  393 

began  in  the  other  hand.  Finally  the  left  arm  began  to  jerk 
with  alternate  contractions  of  the  biceps  and  triceps.  The 
whole  seizure  lasted  more  than  five  minutes.  During  the 
seizure  there  was  dizziness  and  pain  in  the  head,  chiefly  on 
the  right  side. 

Diagnosis :  The  attention  is  at  once  arrested  by  the  data 
of  the  seizures  described.  It  appeared  that  we  had  to  as- 
sume an  irritation  of  the  right  side  of  the  brain,  possibly  due 
to  vascular  disease,  or  to  brain  tumor,  or  perhaps  to  syphilis. 
The  shock  with  residual  hemiplegia  would  be  consistent 
enough  with  any  of  these  diagnoses.  However,  the  history 
seemed  somewhat  long  for  brain  tumor.  Nor  were  there 
any  definite  symptoms  of  intracranial  pressure.  "Adequate" 
treatment  unfortunately  does  not  rule  out  syphilis.  The 
comparatively  early  age  (39)  of  the  patient  makes  it  difficult 
to  explain  the  vascular  disease  except  on  the  basis  of  syphilis. 
Add  to  the  hemiplegia  the  euphoria  and  grandiose  ideas  of  a 
year's  duration,  and  we  arrive  at  a  diagnosis  of  neurosyphilis, 
probably  PARETIC  NEUROSYPHILIS. 

The  laboratory  tests  showed  the  W.  R.  of  the  serum  and 
spinal  fluid  positive,  80  cells  per  cmm.  in  the  fluid,  large 
amounts  of  globulin  and  albumin,  and  a  "  paretic  "  type  of 
gold  sol  reaction. 

To  be  sure  the  Jacksonian  seizure  is  not  especially  charac- 
teristic of  paretic  neurosyphilis,  and  even  suggests  a  local 
irritation  in  the  motor  area,  such  as  a  localized  meningitis, 
possibly  of  a  diffuse  gummatous  nature. 

This  patient  was  put  on  intensive  antisyphilitic  treat- 
ment, namely,  salvarsan  twice  a  week  and  injections  of 
mercury.  He  recovered  rapidly.  After  a  few  months  he 
left  the  hospital,  and  after  treatment  had  continued  for  a 
year,  he  resumed  his  work  by  which  time  both  blood  and 
spinal  fluid  had  become  negative. 

It  must  be  recalled  that  this  patient  had  from  the  time  of 
his  infection  what  has  been  considered  good  antisyphilitic 
therapy,  in  spite  of  which  he  developed  after  a  period  of 
years,  the  symptoms  and  signs  of  neurosyphilis  in  its  most 
dangerous  form.  The  conclusion  must  be  drawn  that 
however  good  such  treatment  is  for  the  majority  of  cases,  it 


394  TREATMENT 

was  insufficient  for  Morovitz.  That  the  early  failure  to  cure 
was  not  due  to  any  "drug- fastness"  of  the  spirochete  or  to  any 
peculiarity  of  strain  is  proved  by  the  result  of  more  vigorous 
antisyphilitic  treatment  which  caused  an  apparent  if  not  a  real 
cure.  With  our  modern  methods  of  treatment  checked  by 
Wassermann  reactions  and  spinal  fluid  examinations,  treat- 
ment is  given  according  to  the  needs  of  the  individual  patient 
rather  than  according  to  general  preconceptions.  We  have 
reason  to  believe  that  under  these  conditions  there  will  be 
fewer  cases  developing  late  symptoms  on  account  of  in- 
sufficient treatment  given  even  to  patients  who  are  willing 
to  co-operate  to  the  last  degree. 

The  fact  that  Morovitz  had  no  apparent  symptoms  for 
several  years  led  to  rather  desultory  treatment  chiefly  in  the 
form  of  mercury  by  mouth.  Previous  to  the  time  when  the 
W.  R.  and  lumbar  puncture  were  available,  the  physician  had 
no  exact  means  of  determining  cure  except  the  non-appearance 
of  symptoms.  But  a  period  of  years  of  quiescence  before 
the  outbreak  of  symptoms  referrable  to  the  involvement  of 
the  nervous  system  is  characteristic  of  syphilis.  With  this 
knowledge  in  mind  it  is  evident  that  today  the  care  of  a 
syphilitic  patient  must  be  guided,  in  part  at  least,  by  examina- 
tions of  the  spinal  fluid  and  W.  R. 


TREATMENT  395 


Salvarsan  treatment  may  even  occasionally  be  of 
value  in  simple  FEEBLEMINDEDNESS  due  to 
congenital  syphilis. 


Case  123.  The  somewhat  unattractive  Robert  Matthews 
was  brought,  at  5  years  of  age,  to  the  hospital  for  backward- 
ness of  mind.  It  appears  that  the  patient  was  born  at  term, 
with  instruments,  that  he  began  to  talk  at  a  year,  and  to 
walk  at  13  months,  but  that  in  point  of  fact,  he  had  not 
talked  intelligibly  to  date.  Robert  had  never  played  with 
other  children  and  is  regarded  by  his  parents  as  backward. 
In  fact,  Robert's  sister  —  a  year  his  junior  —  is  much 
brighter.  Robert  had  had  scarlet  fever  but  without  sequelae. 

Examination  by  the  Binet  scale  showed  that,  although  he 
is  actually  5^  years,  he  graded  by  the  Binet  scale  at  4  and 
was  regarded  as  feebleminded. 

The  physical  examination  showed  a  general  adenopathy 
and  prominent  frontal  bosses.  In  the  study  of  the  family 
history  in  the  search  for  an  etiology  for  the  evident  feeble- 
mindedness, little  or  none  could  be  found.  There  were  no 
miscarriages  or  stillbirths;  the  parents  were  living  and  well. 
There  was  only  the  one  sister  above-mentioned,  who  is 
brighter  than  Robert. 

The  advantage  of  a  routine  W.  R.  is  here  well  shown,  for 
the  W.  R.  in  the  serum  was  positive. 

1.  What  is  the  prognosis  of  cases  of  syphilitic  feebleminded- 

ness? It  would  appear  that  every  case  is  an  individual 
problem. 

2.  What  is  the  effect  of  treatment?     Robert  Matthews  was 

given  mercury  protoiodid  |  gr.,  three  times  a  day,  by 
mouth,  for  three  months.  The  protoiodid  was  followed 
by  ten  injections  of  salvarsan,  average:  0.15  gram, 
during  six  months.  At  the  end  of  this  period,  the  W.  R. 
in  the  blood  had  become  negative.  A  re-examination  by 
the  Binet  scale,  when  Robert  was  6^  years  of  age, 
showed  him  to  grade  at  sf ,  so  that  one  might  conclude 
that  Robert  had  shown  more  mental  progress  in  a  year 
than  he  had  previously. 


396  TREATMENT 

Note:  The  patient's  sister,  4  years  of  age,  is  attractive 
and  bright,  measuring  beyond  her  actual  age  according  to 
the  intelligence  tests.  However,  the  girl  was  found  to  have 
a  positive  W.  R.  It  may  be  that  Robert  and  his  sister 
illustrate  the  hypothesis  of  Mott:  that  the  syphilitic  virus 
becomes  less  potent  as  the  years  go  on,  and  that  the  younger 
children  in  the  family  are  less  affected  than  the  older.  How- 
ever, in  our  series,  there  are  a  number  of  instances  in  which 
this  hypothesis  is  not  substantiated. 

3.  What  is  the  share  of  syphilis  in  the  production  of  feeble- 
mindedness? The  percentage  of  syphilitic  cases  found 
in  institutions  is  not  high.  A  variety  of  cases  have 
been  proveu  to  be  congenitally  syphilitic  in  the  absence 
of  a  positive  serum  W.  R. 

Fernald*  has  charted  a  comparison  of  cases  diagnosti- 
cated "moron"  (that  is,  feeblemindedness  proper,  in  the 
narrower  English  sense)  and  "imbecile."  Fernald  says  that 
the  morons  have,  as  a  group,  many  more  bad  family  histories 
than  have  the  imbeciles,  to  quote  —  "Only  70%  of  the 
[imbecile]  group  have  bad  family  histories.  This  at  first 
seems  surprising,  but  when  we  consider  that  more  of  our 
syphilitic,  traumatic,  and  sporadic  cases  tend  toward  the 
lower  end  of  the  feebleminded  group,  and  when  we  remember 
that  with  such  cases  there  is  often  a  seemingly  normal  family 
tree,  the  drop  in  the  curve  appears  logical." 

The  situation  with  the  idiots,  of  whom  only  38  came  into 
Fernald's  study,  was  similar;  12  out  of  38,  or  32%,  of  idiots, 
had  good  family  histories.  On  these  figures,  how  unfortu- 
nate it  would  be  to  dub  feeblemindedness  hereditary!  It  is 
true,  however,  that  68-70%  of  the  idiots  and  imbeciles, 
judging  by  W.  E.  Fernald's  intensive  study,  do  have  bad 
family  histories. 

Goddard  f  states  that  of  all  the  causes  of  feeblemindedness, 

*  Fernald,  W.  E.  Standardized  Fields  of  Inquiry  for 
Clinical  Studies  of  Borderline  Defectives.  Mental  Hygiene, 
Vol.  i,  No.  2,  April,  1917. 

t  Goddard,  H.  H.,  Feeblemindedness,  its  Causes  and 
Consequences,  1914. 


TREATMENT  397 

there  is  perhaps  none  for  which  there  is  less  evidence  than 
syphilis.  Goddard  found  syphilis  in  27  of  his  intensively 
charted  cases  of  feeblemindedness,  that  is,  in  9%  of  all  his 
charts.  He  finds  the  majority  of  the  syphilis  cases  occurring 
in  relatives  of  the  feebleminded  to  be  in  the  hereditary  group ; 
for  example,  of  164  charts  in  the  hereditary  group,  17,  or  10%, 
showed  syphilis.  In  34  charts  in  a  group  termed  "probably 
hereditary"  3,  or  9%,  showed  syphilis.  Of  37  charts  in  the 
group  termed  "neuropathic"  4,  or  n%,  showed  syphilis, 
whereas  in  57  "accident"  and  8  "no  cause"  groups,  there 
were  but  2  (4%),  and  one,  or  13%,  showing  syphilis.  How- 
ever, Goddard  concedes  that  much  more  careful  studies  are 
necessary  if  we  are  to  give  an  exact  evaluation  of  syphilogenic 
feeblemindedness. 

The  first  ten  of  the  Waverley  Anatomical  Series  are  shortly 
to  be  described  in  a  forthcoming  publication.*  Of  these  ten 
cases,  four  showed  some  slight  evidence  of  chronic  inflam- 
matory changes,  indicating  the  possibility  of  a  syphilitic  or 
similar  infectious  condition.  These  cases,  be  it  remembered, 
were  not  cases  of  juvenile  paresis,  but  cases  of  what,  for  the 
lack  of  a  better  name,  may  be  called  "ordinary"  feeble- 
mindedness. 

If  all  or  any  of  these  processes  are  syphilitic,  the  syphilis  is 
virtually  extinct.  The  cases  had  not  been  treated  for  syphilis 
and  were  not  regarded  as  syphilitic,  though  several  of  them 
showed  a  few  stigmata  somewhat  suggestive  of  syphilis.  The 
anatomical  conclusion  at  this  time  is  still  doubtful. 

As  in  the  text  case,  the  hypothesis  of  syphilis  as  a  direct 
cause  for  simple  feeblemindedness  must  be  entertained  for  a 
few  cases.  In  any  event,  it  would  not  seem  logical  to  let 
any  institution  for  the  feebleminded  run  without  a  Wasser- 
mann  analysis  of  the  population.  In  addition  to  the  Wasser- 
mann  data  from  the  blood  serum,  osteological  data  from  the 
X-ray  have  proved  of  occasional  value  for  syphilis  diagnosis 
in  this  as  in  other  groups. 

*  W  E  Fernald  and  E.  E.  Southard.  Waverley  Research 
Series  in  the  Pathology  of  the  Feebleminded.  Proceedings 
of  the  American  Academy  of  Arts  and  Sciences,  1917. 


1  Within  the  gates  of  Hell  sat  Sin  and  Death." 

Paradise  Lost,  Book  X,  Line  230. 


VI.   NEUROSYPHILIS  AND  THE  WAR. 

Although  the  American  toll  of  war  syphilis  has  not  yet 
begun  and  although  the  crop  of  neurosyphilis  due  to  war 
infections  may  not  arrive  until  the  mid  or  late  twenties  of 
the  century  (witness  German  experience  in  the  eighties  of 
the  last  century),  it  seems  proper  here  to  give  a  number  of 
abstracts  re  neurosyphilis  as  it  has  developed  in  the  war. 
Available  reports  from  English,  French,  and  German  sources 
have  been  levied  upon  for  the  years  1914-16. 

It  is  clear  that  all  the  armies  have  had  their  share  of 
neurosyphilitics,  some  clearly  diseased  before  enlistment, 
some  developing  symptoms  as  a  result  of  training,  stress, 
or  shock,  others  hastened  or  made  worse  by  war  conditions. 

There  are  important  questions  of  pension,  retirement,  and 
compensation  for  neurosyphilitics.  No  previous  war  has 
had  the  benefit  of  the  Wassermann  reaction  and  other  exact 
tests  bearing  upon  the  nature,  progress,  and  curability  of 
neurosyphilis. 

That  we  shall  have  our  fill  of  pension  and  other  problems 
can  already  be  seen  from  continental  reports.  Thibierge,* 
for  example,  states  that  syphilis  has  become  a  real  epidemic 
among  the  French  soldiers  and  mobilized  munition  workers. 

Hechtf  of  Austria  claims  that  no  less  than  an  equivalent 
of  60  army  divisions  have  been  temporarily  withdrawn  from 
fighting  on  the  Teutonic  side  for  venereal  diseases.  He 
commends  Neisser's  idea  that  salvarsan  and  mercury  should 
be  given  in  the  trenches.  While  hundreds  or  thousands  of 
Austrians  are  sick  with  syphilis,  sound  and  healthy  men  are 
being  shot  down  in  their  stead.  The  diagnosis  of  syphilis, 
according  to  Hecht,  ought  to  be  a  signal  for  sending  the  men 
to  the  front.  He  makes  even  the  somewhat  bizarre  sugges- 

*  Thibierge.     La  Syphilis  dans  1'armee,  1917. 
f  Hecht.     Wien.  klin.  Woch.,  xxix,  51. 

399 


400  NEUROSYPHILIS  AND   THE  WAR 

tion  that  special  companies  of  syphilitics  should  be  formed, 
for  convenience  of  treatment,  on  the  firing  line. 

Not  only  is  the  syphilis  problem  in  the  army  of  importance 
to  the  military  authorities,  but  also  to  the  civil  population, 
and  perhaps  to  them  a  greater  problem.  With  the  great 
increase  of  venereal  disease  that  is  the  result  of  the  conditions 
of  army  life  in  war  time,  there  will  be  a  considerable  percent- 
age of  cases  developing  neurosyphilis  a  number  of  years  after 
discharge  from  the  army,  but  caused  by  the  infection  acquired 
during  service.  In  addition  many  men  will  bring  the  disease 
back  to  America  in  an  infectious  stage  and  spread  it.  We 
would  advocate  that  the  names  of  all  soldiers  who  had 
acquired  syphilis  and  were  not  considered  cured  at  time  of 
discharge  should  be  given  to  health  organizations  in  their 
home  states  that  they  may  be  given  further  care. 

These  practical  and  several  theoretical  questions  are 
raised  by  the  following  fourteen  cases  which  we  have  con- 
densed from  their  sources. 


NEUROSYPHILIS  AND  THE  WAR  40! 


A  tabetic  lieutenant  "shell-shocked"  into  paresis? 
Case  from  Donath  of  Vienna. 


Case  A.*  An  apparently  competent  German  professor  in 
an  intermediate  school,  a  lieutenant  of  infantry  reserves,  33 
years  old,  on  the  I7th  August,  1914,  was  stunned  for  a  while 
by  the  shock  of  a  cannon-firing  25  feet  away.  Urination 
became  difficult.  Headaches  and  limb  pains  ensued,  with 
paralysis  of  fingers,  gastric  troubles,  forgetfulness  especially 
for  names,  insomnia,  and  general  scattering  of  mental  facul- 
ties. 

Neurologically,  the  pupils  were  irregular,  left  larger  than 
right;  Argyll- Robertson  reaction.  Right  knee-jerk  livelier 
than  left.  Achilles  reactions  absent.  Slow  and  dissociated 
pain  reactions  in  feet,  lower  thighs  and  lower  quarter  of  upper 
thighs,  with  hypalgesia  or  analgesia.  Station  good;  gait 
steady.  Mentally  depressed,  slow  of  thought.  Speech  poor 
and  of  indistinct  construction  (mild  dementia).  Calculation 
ability  poor.  No  pleasure  in  work. 

Wassermann  reaction  of  serum  weakly  positive. 

It  seems  that  for  a  year  the  patient  had  been  subject  to 
spells  of  anger.  He  was  irritated  by  his  wife  who  had  been 
nervous  since  an  earthquake. 

On  the  occasion  of  the  earthquake,  1911,  the  patient  himself 
had  had  a  spell  of  difficulty  with  urination.  The  spell  had 
lasted  two  or  three  months.  The  patient  had  had  a  chancre 
in  1902,  "cured"  in  four  or  five  weeks  with  xeroform.  In 
1908,  when  about  to  marry,  he  had  had  six  mercurial  inunc- 
tions. 

I.  Is  this  a  case  of  traumatic  paresis?  From  the  some- 
what meagre  account  it  would  appear  that  Donath's 
lieutenant  should  rather  be  termed  "shell-shock  pare- 
sis," in  the  sense  of  a  paretic  neurosyphilis  liberated  by 

»  —  " 

*  Donath.  Beitrage  zu  den  Kriegsverletzungen  und  -er- 

krankungen  des   Nervensystems.     Wiener  klin.  Wchnschr., 

No.  27-8,  1915. 


402  NEUROSYPHILIS  AND  THE  WAR 

shell-shock  (using  shell-shock  in  the  sense  of  a  shock 
without  direct  brain  injury). 

2.  What  compensation  is  due  such  a  man  as   Donath's 

lieutenant?  The  ordinary  principles  applicable  to 
traumatic  paresis  are  not  here  in  point,  since  no 
symptoms  pointing  to  trauma  of  brain  ever  super- 
vened. See  discussion  under  Case  G. 

3.  How  frequent  is  paresis  in  armies?     R.  L.  Richards  in 

White  and  Jelliffe's  Treatment  of  Nervous  and  Mental 
Diseases  writes  as  follows  (of  course  concerning  peace 
times) : 

"The  French  estimate  that  paresis  cases  are  7  per 
cent  of  all  their  military  cases.  The  German  estimate 
is  6.6  per  cent.  In  our  own  army  at  the  Government 
Hospital  for  the  Insane,  of  490  cases  of  mental  diseases 
among  officers  and  enlisted  men,  37,  or  7  per  cent,  were 
paresis.  During  the  Russo-Japanese  War,  in  the 
Russian  Psychiatric  Hospital  at  Harbin,  the  percentage 
of  paresis  was  5.6  per  cent  among  the  cases  developing 
at  the  front." 


NEUROSYPHILIS  AND  THE  WAR  403 


A  French  soldier  "shell-shocked"  (also  burial) 
into  incipient  tabes  dorsalis?  Case  from  Duco  and 
Blum  of  Paris. 


Case  B.*  A  French  soldier  was  buried  by  effects  of  shell 
explosion  September  8th,  1914.  He  sustained  no  wound  or 
fracture. 

Incontinence  of  urine  developed.  Anesthesia  of  penis  and 
scrotum.  Reflexes  absent;  pupils  sluggish.  Wassermann 
reactions  suspicious. 

The  diagnosis  tabes  dorsalis  incipiens  was  made  (hema- 
tomyelia  of  conus  terminalis  eliminated). 

The  patient  was  estimated  to  be  "40%  incapacitated," 
according  to  the  French  "  echelle  de  gravite"  of  conditions.  A 
full  pension  would  not  be  justified  in  the  opinion  of  the 
French  authors. 

I.  Is  there  evidence  of  an  increase  or  exacerbation  of 
tabes  dorsalis  in  the  war?  Birnbaum,f  reviewing 
German  war  neurology,  quotes  Weygandt  as  believing 
that  the  war  has  probably  had  to  do  with  the  produc- 
tion of  both  tabes  and  paresis  in  many  instances. 
Other  cases,  however,  have  merely  been  made  worse 
by  the  war  stress.  Thirdly,  there  are  cases  in  which 
the  war  stress  has  done  no  harm  whatever.  Westphal 
has  seen  both  tabes  and  paresis  develop  in  men  who 
had  never  before  shown  any  mental  or  physical  symp- 
toms whatever,  and  accordingly,  Westphal  must  be 
counted  among  those  who  regard  war  stress  as  a  liberat- 
ing factor  for  these  diseases.  Redlich  and  Donath  are 
cited  in  the  same  connection.  (The  case  of  Donath  is 
the  case  presented  above  as  Case  A.) 

A  very  interesting  claim  was  made  by  Cimbal  to  the 
effect  that  he  found  many  examples  of  paresis  develop- 
ing in  the  early  period  of  the  war,  particularly  in  Novem- 
ber and  December,  1914.  Later,  according  to  Cimbal, 
cerebrospinal  syphilis  and  tabes  became  more  prevalent. 

*  Duco  et  Blum.  Guide  pratique  du  Medecin  dans  les 
Expertises  medicol6gales  militaires.  Paris,  1917. 

t  Birnbaum.  Kriegsneurosen  und  -psychosen  auf  Grund 
der  gegenwartigen  Kriegsbeobachtungen :  Sammelbericht. 
Z.  f.  d.  ges.  Neurol.  u.  Psychiat.,  Bd.  XII,  H.  I,  1915. 


404  NEUROSYPHILIS  AND  THE  WAR 


Neurosyphilis  in  a  German  recruit,  possibly  AG- 
GRAVATED ON  military  SERVICE.  Pension  not 
allowable.  Case  from  Weygandt. 


Case  C.*  A  German,  long  alcoholic  and  thought  to  be 
weakminded,  volunteered,  but  shortly  had  to  be  released  from 
service.  He  began  to  be  forgetful  and  obstinate,  cried,  and 
even  appeared  to  be  subject  to  hallucinations.  The  pupils 
were  unequal  and  sluggish.  The  uvula  hung  to  the  right. 
The  left  knee-jerk  was  lively,  right  weak.  Fine  tremors  of 
hands.  Hypalgesia  of  backs  of  hands.  Stumbling  speech. 
Attention  poor. 

It  appeared  that  he  had  been  infected  with  syphilis  in  1881 
and  in  1903  had  had  an  ulcer  of  the  left  leg. 

The  military  commission  denied  that  his  service  had 
brought  about  the  disease.  In  the  phrase  of  the  Canadian 
Pension  Board  the  German  commission  would  probably  have 
rendered  a  report  "aggravated  on  service,"  not  "by  service." 
(See  Canadian  cases  D,  E,  and  F.) 

1.  Has  paresis  increased  in  the  war?    Both  French  and 

German  figures  controvert  the  claim.  Marie,  for 
example,  found  not  a  single  paretic  amongst  the  skull 
injury  cases  at  the  Salpetriere.  Most  authors  are 
found  demonstrating  cases  which  they  clearly  regard  as 
in  some  way  produced  or  unfavorably  influenced  by 
the  war.  There  seems,  therefore,  to  be  a  little  incon- 
sistency between  the  general  statement  that  paresis  has 
not  increased  in  the  war  and  the  somewhat  frequent  cases 
described  as  occurring  in  and  modified  by  the  war. 
However,  Bonhoeffer,  on  the  basis  of  nine  months'  war 
experience,  also  holds  it  to  be  probable  that  paresis  is 
no  more  frequent  in  the  field  than  in  the  home  popula- 
tion. 

2.  Is  the  old  syphilitic  especially  liable  to  break  down  under 

war  conditions?  According  to  Richards,  Shaikewicz 
says  that  in  the  Russo-Japanese  war  paresis  was  noted 

*  Weygandt.  Kriegseinfliisse  und  Psychiatric.  Jahres- 
kurse  f.  arztl.  Fortbildung,  Maiheft,  1915. 


NEUROSYPHILIS  AND  THE  WAR  405 

especially  among  the  officers  and  non-commissioned 
officers,  and  that  it  was  undoubtedly  hastened  in  its  de- 
velopment by  war  conditions.  Steida  says  that  while 
ordinarily  we  find  paresis  developing  twelve  to  twenty 
years  after  the  primary  sore  of  syphilis,  in  these  cases  it 
developed  in  five  to  ten  years  after  the  primary  sore. 
Some  of  the  cases  progressed  with  unusual  rapidity.  It 
was  also  noticed  that  among  soldiers  from  the  front, 
under  treatment,  evidences  of  syphilis  were  present  in 
20%,  while  among  the  other  soldiers  under  treatment, 
evidences  of  syphilis  were  present  in  1.6%.  Undoubt- 
edly the  old  syphilitic  is  especially  liable  to  break 
down  under  war  conditions. 

But,  on  the  whole,  the  German  authors  in  this  war 
find  no  evidence  favoring  Steida's  claim  of  the  hastened 
post-infective  outbreak. 

How  did  it  come  about  that  the  efficient  German  system 
permitted  this  alcoholic  and  weakminded  syphilitic  to 
enter  the  army?  As  will  be  seen,  he  was  a  volunteer. 
In  general,  the  German  system  has  been  supplied  with 
army  surgeons  who  have  been  trained,  not  by  brief  and 
"brush-up"  courses,  but  by  longer  periods,  sometimes 
two  years  in  duration. 


406  NEUROSYPHILIS  AND  THE  WAR 


Syphilis  contracted  before  enlistment,  "AGGRA- 
VATED BY  SERVICE."  Canadian  case,  courtesy 
of  Dr.  J.  L.  Todd,  Canadian  Board  of  Pension 
Commissioners. 


Case  D.  A  laboring  man,  42,  who  always  strenuously 
denied  syphilitic  infection,  proceeded  to  France  eight  months 
after  enlistment.  He  had  not  been  in  France  three  weeks 
when  he  dropped  unconscious.  He  regained  consciousness, 
but  remained  stupid,  dull  in  expression,  and  with  memory 
impaired.  His  speech  was  also  impaired.  There  was  dizzi- 
ness and  a  right-sided  hemiplegia. 

He  was  confined  to  bed  four  months  and  was  then 
"boarded"  for  discharge. 

Physically,  his  heart  was  slightly  enlarged  both  right  and 
left;  sounds  irregular;  extra  systoles ;  aortic  systolic  murmur 
transmitted  to  neck;  blood  pressure  140:40.  Precordial 
pain,  dyspnoea. 

Neurologically,  there  was  a  partial  spastic  paralysis  of  the 
right  thigh  which  could  be  abducted,  could  be  flexed  to  120°, 
and  showed  some  power  in  the  quadriceps.  There  was  also 
a  spastic  paralysis  of  the  right  arm,  but  the  shoulder  girdle 
movements  were  not  impaired.  There  was  a  slight  weakness 
on  the  right  side  of  the  face.  There  was  no  anesthesia 
anywhere. 

The  deep  reflexes  were  increased  on  the  right  side,  Babinski 
on  right,  flexor  contractures  of  right  hand,  extensor  contrac- 
tures  of  right  leg,  abdominal  and  epigastric  reflexes  absent, 
pupils  active,  tongue  protruded  in  straight  line. 

Fluid :   slight  increase  in  protein.     W.  R.  +  +  + 

The  Board  of  Pension  Commissioners  ruled  that  the  condi- 
tion had  been  aggravated  by  service.  (See  Case  E,  "aggra- 
vated on  service.") 

I.  In  view  of  the  fact  that  the  majority  of  the  cases  here 
abstracted  happen  to  be  in  common  soldiers,  is  there 
any  evidence  bearing  on  relative  incidence  in  officers 
and  men?  Quoting  R.  L.  Richards: 


NEUROSYPHILIS  AND  THE  WAR  407 

"The  percentage  of  paresis  cases  among  officers  alone 
is  variously  estimated  from  50  per  cent  in  the  German 
army  (Stier)  to  58.9  per  cent  in  the  Austrian  army 
(Drastich) .  Since  paresis  is  a  disease  of  more  advanced 
life,  it  is  but  natural  that  the  percentage  of  paresis 
among  officers,  non-commissioned  officers,  and  older 
soldiers  should  be  higher  than  among  the  whole  military 
body,  where  the  average  age  is,  as  we  have  seen,  well 
below  thirty  years.  Hence  the  above  figures  do  not 
mean  a  greater  prevalence  of  syphilis  among  those 
classes,  but  that  we  have  no  means  of  knowing  how 
many  of  the  others  develop  paresis.  If  anything  it 
shows  that  these  'soldiers  by  calling,'  have  a  more 
stable  mental  make-up,  since  they  succumb  chiefly  to 
an  exogenous  toxin." 

Rayneau  at  the  igth  Congress  of  French  Alienists 
and  Neurologists  at  Nantes  in  1909,  discussing  the 
insane  of  the  army  from  a  medico-legal  point  of  view, 
states  that  the  most  frequent  mental  disease  amongst 
officers  and  soldiers  is  general  paresis.  At  least,  this 
disease  is  the  most  frequent  basis  of  invaliding,  retire- 
ment, or  placing  in  the  inactive  list.  He  states  that 
French  and  foreign  statistics  are  at  one  upon  this 
matter,  quoting  Christian  as  finding  32%  among  the 
soldiers  interned  at  Charenton;  Gamier  at  Dijon, 
59%;  Meilhon  at  Quimper,  42%  and  Talon  at  Mar- 
seilles, 33.8%.  Grilli  found  31  of  40  officers  interned 
in  Florence,  Sienna  and  Milan  victims  of  general 
paresis.  Stier's  German  statistics  indicate  about  50%. 
Rayneau  himself  found  1 6  of  20  officers  paretic  and  17 
out  of  27  subalterns  and  gendarmes. 

The  Neurological  Society  of  Paris  held  a  conference 
December  15,  1916,  with  the  chiefs  of  the  neurological 
and  psychiatric  military  centres  of  France,  and  dis- 
cussed a  variety  of  questions  concerning  invaliding, 
incapacity,  and  compensation  in  neuroses  and  psy- 
choses of  war.  Dupr6  dealt  especially  with  the  psy- 
choses of  war  as  caused  by  trauma,  strain,  infection, 
and  intoxication.  General  paresis  is  regarded  by 
Dupr£  as  the  most  important  of  the  dementias  found  in 
the  army.  The  medico-legal  point  of  view  is,  of  course, 
that  general  paresis  is  necessarily  related  to  an  old 
syphilis,  but  its  late  development  leads  to  misinterpre- 
tations as  to  its  probable  cause,  both  by  the  family  and 
friends  and  even  by  magistrates.  The  war  acts  in 
the  French  nomenclature  as  an  agent  revelateur  or  as  an 


408  NEUROSYPHILIS   AND   THE   WAR 

agent  accSlerateur.  Although  its  cause  is  prior  and 
exterior  to  the  war,  general  paresis  in  a  majority  of 
cases  is  brought  out  (revele)  by  the  lack  of  adaptability 
of  the  general  paretic  to  the  novelty  and  difficulties  of 
his  surroundings  and  duties  in  war.  Trauma,  strain, 
and  alcohol  in  a  certain  number  of  cases  accelerate  the 
progress  of  a  general  paresis.  The  aggravation  of 
paresis  is  produced  by  these  same  factors,  but  especially 
by  violent  cerebral  trauma.  According  to  Dupre,  the 
Val-de-Grace  statistics  show  that  the  number  of  pa- 
retics  has  not  been  increased  by  the  war.  Medico- 
legally,  the  victim  of  general  paresis,  like  the  victim  of 
traumatic  or  infectious  chronic  mental  disorder,  may 
be  assigned  an  incapacity  of  from  50  to  100%,  and 
these  patients  are  invalided  under  Reforme  No.  I,  —  a 
permanent  invaliding. 

Lupine  of  Lyons  also  discusses  the  compensation 
question  in  general  paresis.  Lupine  thinks  that,  al- 
though syphilis  is  indispensable  in  paresis,  yet  the  truth 
is  that  syphilis  plus  something  else  unknown  to  us  is 
responsible  for  general  paresis.  This  something  else  is 
neither  a  special  kind  of  virus  nor  is  it  a  particular  kind 
of  prepared  soil  alone.  Trauma,  physical,  intellectual, 
and  moral  strain,  and  insomnia  are  the  factors  to  which 
he  calls  special  attention  as  adjuncts  in  the  production 
of  general  paresis.  As  to  the  responsibility  of  the  State 
for  the  production  of  general  paresis,  according  to 
L6pine,  the  maximal  responsibility  should  be  40%  on 
account  of  the  very  considerable  predisposition  to  paresis 
created  by  pre-existent  syphilis. 

Marie  remarked  that,  although  there  had  been 
thousands  of  head  cases  at  the  Salp£tri£re,  there  had 
not  been  a  single  case  of  general  paresis.  Dupre  agreed 
with  Marie  that  trauma  was  not  a  frequent  etiological 
factor;  strain  and  alcohol  were  more  important.  The 
Society  agreed  that  in  exceptional  cases,  where  an 
encephalic  trauma  could  be  regarded  as  accelerating 
°r.  aggravating  the  disease,  the  degree  of  incapacity 
might  be  set  at  from  10  to  30  per  cent. 


NEUROSYPHILIS  AND  THE  WAR  409 


Syphilis  contracted  before  enlistment,  "  AGGRA- 
VATED ON  SERVICE."  Canadian  case,  courtesy 
of  Dr.  J.  L.  Todd,  Canadian  Board  of  Pension 
Commissioners. 


Case  E.  A  laboring  man,  44,  acquired  syphilis  at  a  time 
unknown.  Ten  months  after  enlistment  this  man  developed 
symptoms  on  the  firing-line.  He  was  inattentive,  irrational, 
incoherent.  The  diagnosis  was  then  "mania." 

There  were,  however,  scars  at  angle  of  mouth  and  on  lower 
lip.  Occipital  glands  were  palpable,  fine  tremor  of  hands. 
TheW.  R.  was  +  +  +. 

Later  the  patient  became  violent,  destructive,  untidy, 
disoriented.  Auditory  hallucinations  are  recorded. 

He  was  "boarded"  for  discharge  five  months  after  the  first 
symptoms.  The  board  agreed  that  these  symptoms  would 
have  appeared  in  civil  life.  In  view  of  a  difference  of  opinion 
as  to  the  part  played  by  stress  of  service,  his  condition  was 
set  down  as  "aggravated  on  service  "  (not,  it  will  be  noted,  by 
service,  see  Case  D). 

1.  Under  what  conditions  should  pensions  be  awarded  for 

disability  resulting  from  venereal  diseases?  According 
to  a  personal  communication  from  Dr.  J.  L.  Todd, 
Chairman  of  the  Board  of  Pension  Commissioners  for 
Canada,  pensions  are  awarded  for  all  disabilities  appear- 
ing during  service,  unless  they  can  be  shown  certainly 
to  be  due  to  the  men's  own  fault  and  negligence.  It 
would  appear  that  during  service  covers  both  aggra- 
vations by  and  on  service.  There  remains  some  doubt 
as  to  whether  contraction  of  venereal  disease  constitutes 
negligence. 

2.  What  have  been  conditions  in  the  small  inactive  American 

army  of  the  past?  Richards  has  made  a  study  of 
statistics  at  the  Government  Hospital  for  the  Insane, 
Washington. 

"The  leading  features  of  this  mental  disease  were 
well  exemplified  in  our  cases  the  past  year.  They 
formed  7.5  per  cent  of  the  total  number.  They  aver- 
aged forty  years  of  age,  and  Ziehen  says  80  per  cent  of 


410  NEUROSYPHILIS  AND  THE  WAR 

all  cases  are  in  the  fourth  or  fifth  decade  of  life.  They 
averaged  ten  and  a  half  years'  service,  which  would 
indicate  that  the  military  life  was  their  calling.  Only 
one  had  any  serious  hereditary  defect.  Stigmata  of 
degeneration  were  infrequent,  averaging  only  two  for 
each  case.  66  per  cent  had  good  schooling,  considering 
their  opportunities.  Physical  signs  were  frequent  in 
each  case.  Only  one  snowed  normal  light  reaction. 
Ziehen  says  the  light  reaction  is  retained  in  only  20 
per  cent  of  the  cases.  Patellar  reflex  was  absent  in  one 
case  and  normal  or  exaggerated  in  five.  The  speech 
defect  was  slight  in  four  cases.  Other  physical  signs 
were  present  in  the  usual  proportions.  Memory  de- 
fects existed  in  all  the  cases.  In  four  the  onset  was 
with  excitement.  One  began  with  a  character  change 
as  the  most  marked  feature.  In  only  two  were  the 
transfer  diagnoses  correct.  One,  beginning  as  a  quiet 
dementia,  was  diagnosticated  paralysis  agitans,  be- 
cause of  a  marked  tremor.  One  was  excited  and 
euphoric  and  was  called  a  manic-depressive  psychosis. 
One  with  an  obscure  onset  was  diagnosticated  as  a 
neurasthenic.  The  other  one  was  first  observed  in  this 
hospital.  The  physical  signs  should  have  led  to  a 
correct  diagnosis  in  each  of  these  cases." 


NEUROSYPHILIS  AND  THE  WAR  4!  I 


Duration  of  neurosyphilitic  process  important  re 
compensation.  Canadian  case,  courtesy  of  Dr. 
C.  B.  Farrar,  Psychiatrist,  Military  Hospitals 
Commission. 


Case  F.  A  Canadian  of  36  enlisted  in  1915,  served  in 
England,  and  was  returned  to  Canada  in  February,  1917, 
clearly  suffering  from  some  form  of  neurosyphilis  (W.  R. 
positive  in  serum  and  fluid,  globulin,  pleocytosis  108). 

There  is  no  record  of  any  disability  or  symptom  of  nervous 
or  mental  disease  at  enlistment.  The  first  symptoms  were 
noted  by  the  patient  in  May,  1916,  six  months  or  more  after 
enlistment.  The  case  was  reviewed  at  a  Canadian  Special 
Hospital,  October  n,  1916,  by  a  board  of  examiners.  This 
board  reported  that: 

"The  condition  could  only  come  from  syphilitic  infection 
of  three  years'  standing"  (a  decision  bearing  on  compensa- 
tion) ;  but  the  general  diagnosis  remained  : 

"  Cerebrospinal  lues,  aggravated  by  service." 

The  picture  which  the  medical  board  regarded  as  of  at  least 
three  years'  standing  was  as  follows: 

History  of  incontinence,  shooting  pains,  attacks  of  syncope, 
general  weakness,  facial  tremor,  exaggerated  knee-jerks, 
pupils  react  with  small  excursion.  Speech  and  writing  dis- 
order, perception  dull,  lapses  of  attention,  memory  defect, 
defective  insight  into  nature  of  disorder,  emotional  apathy. 

1.  Was   the   conclusion   "aggravated   by  service "    sound? 

On  humanitarian  grounds  the  victim  is  naturally  con- 
ceded the  benefit  of  the  doubt.  But  it  is  questionable 
how  scientifically  sound  the  conclusion  really  was. 

2.  Could  the  condition  come  only  from  syphilitic  infection 

of  at  least  three  years'  standing?  Hardly  any  single 
symptom  in  this  case  need  be  of  so  long  a  standing;  yet 
the  combination  of  symptoms  seems  by  very  weight  of 
numbers  to  justify  the  conclusion  of  the  medical  board. 


412  NEUROSYPHILIS   AND   THE   WAR 


Can  PARETIC  NEUROSYPHILIS  ("general 
paresis  ")  be  lighted  up  by  the  stress  of  military 
service  without  injury  or  disease?  A  possible 
example  from  P.  Marie,  Chatelin  and  Patrikios 
of  Paris. 


Case  G.  In  apparently  good  health  a  French  soldier 
repaired  to  the  colors,  in  August,  1914,  being  then  23  years 
old. 

Two  years  later,  August,  1916,  symptoms  appeared: 
speech  disorder  with  stammering,  change  of  character  (had 
become  easily  excitable),  stumbling  gait.  He  became  more 
and  more  preoccupied  with  his  own  affairs,  grew  worse,  and 
was  sent  to  hospital  in  October,  1916. 

He  was  then  foolish  and  overhappy,  especially  when  inter- 
viewed. There  was  marked  rapid  tremor  of  face  and  tongue. 
Speech  hesitant,  monotonous,  and  stammering  to  the  point 
of  unintelligibility.  His  memory,  at  first  preserved,  became 
impaired  so  that  half  of  a  test  phrase  was  forgotten.  Simple 
addition  was  impossible  and  fantastic  sums  would  be  given 
instead  of  right  answers;  handwriting  tremulous,  letters  often 
missed,  others  irregular,  unequal,  and  misshapen. 

Excitable  from  onset,  the  patient  now  became  at  times 
suddenly  violent,  striking  his  wife  without  provocation. 
After  visit  at  home,  he  would  forget  to  return  to  hospital. 
Often  he  would  leave  hospital  without  permission  (of  course 
the  more  surprising  in  a  disciplined  soldier). 

No  delusions  were  found. 

The  serum  and  fluid  W.  R.  were  positive,  albumin  in  fluid, 
lymphocytosis. 

Neurological  examination.  Unequal  pupils,  slight  right- 
side  mydriasis,  pupils  stiff  to  light,  weakly  responsive  in 
accommodation,  reflexes  lively,  fingers  tremulous  on  exten- 
sion of  arms. 

The  patient  had,  December  5,  1916,  an  epileptiform  attack 
with  head  rotation,  limb-contractions  and  clonic  movements. 


NEUROSYPHILIS  AND  THE  WAR  413 

1.  Should   this  soldier  recover  for   disability  obtained^  in 

service?  Marie  was  inclined  to  think  military  service 
in  part  responsible  for  the  development  of  the  paresis. 
Laignel-Lavastine  thought  so  also,  but  that  the 
amount  assigned  should  be  5%-io%  of  the  maximum 
assignable. 

2.  What  is  the  duty  of  the  military  authorities  relative  to 

socalled  traumatic  paresis?  Medico -legally  speaking, 
Froissart,  quoted  by  Rayneau,  states  that  a  victim  of 
traumatic  paresis  may  or  may  not  have  presented  mental 
disorders  before  the  accident,  that  is,  that  the  paretic 
symptoms  may  develop  out  of  a  clear  sky  as  a  result 
of  the  accident.  The  accident  itself  must  be  of  a 
serious  nature.  The  accident  must  be  followed  by 
phenomena  pointing  to  brain  injury  of  traumatic 
nature.  These  phenomena  need  not  be  characteristic 
symptoms  of  general  paresis  at  the  outset.  The  period 
elapsing  between  the  trauma  and  the  supervening  con- 
dition of  paresis  must  be  occupied  without  notable 
interruption,  at  first  by  phenomena  of  a  purely  trau- 
matic nature,  later  by  signs  indicating  the  onset  and 
evolution  of  general  paresis. 

The  French  invaliding  process  called  Reforme  No.  i 
with  pension  is  granted  according  to  the  governmental 
instructions  only  to  officers,  subalterns,  and  soldiers 
whose  disease  is  due  to  trauma.  In  view  of  this 
governmental  regulation,  the  military  surgeon  must 
write  out  certificates  describing  every  cranial  trauma, 
however  slight,  which  might  have  a  bearing  on  the 
development  of  paresis.  However,  he  should  not  too 
readily  admit  trauma  as  a  cause  of  paresis.  If  a  long 
period  of  quietude,  a  period  in  which  the  trauma  itself 
seems  to  have  undergone  a  complete  recovery,  super- 
venes, then  general  paresis  should  not  be  reported  by 
the  surgeon. 

L6pine  has  recently  noted  the  following  features  as 
desirable  in  board  reports  concerning  paretics:  nature 
of  trauma,  length  of  service,  fatigue  endured,  insomnia, 
date  of  infection,  treatment,  W.  R. 


414  NEUROSYPHILIS  AND  THE  WAR 


Can  "  gassing  "  light  up  a  paresis?    Example  from 
de  Massary  of  Issy-les-Moulineaux. 


Case  H.  A  soldier,  35,  was  sent  to  the  Centre  Neurologique 
with  a  hospital  ticket  reading : 

"Neurasthenia,  general  weakness  following  intoxication 
by  gas." 

The  soldier  was  thought  at  first  to  be  a  neurasthenic.  But 
he  soon  showed  signs  of  more  pronounced  mental  trouble. 
The  voice  was  suspicious.  There  was  a  slight  irregularity  of 
pupils. 

An  epileptiform  attack  occurred,  followed  by  aggravation 
of  symptoms. 

Lumbar  puncture  showed  pleocytosis.  The  W.  R.  of  the 
serum  proved  positive. 

Yet  the  evident  neurosyphilis,  possibly  paretic  (de  Mas- 
sary's  diagnosis),  was  preceded  by  a  neurasthenia  and  the 
neurasthenia  was  preceded  by  "gassing." 

De  Massary  believes  the  patient  and  his  family  would 
perhaps  be  justified  in  believing  the  condition  produced  by  the 
injury.  De  Massary  is  not  clear  as  to  the  financial  deserts  of 
the  patient.  It  is  not  a  manifest  case  of  aggravation  of  ante- 
bellum symptoms,  even  if  it  be  neuropathologically  an  in- 
stance of  acquired  loss  of  resistance  to  pre-existent  spirochetes 
in  body  or  brain. 

I.  What  adjuvant  factors  have  been  recognized  in  military 
paresis?  Aside  from  syphilis,  Rayneau  finds  that  alco- 
holism, malaria,  sunstroke  and  various  intoxications 
serve  as  causes  for  paresis.  Rayneau  points  out  that  the 
apparent  integrity  of  the  mind  in  general  paresis  may 
be  such  that  they  last  in  the  army  some  time  and  have 
their  oddities  ascribed  to  misconduct  or  breaches  of 
discipline.  In  fact  the  Legrande  du  Saulle  called  this 
early  period  in  general  paresis  the  medico-legal  period, 
showing,  as  it  so  often  does,  thefts,  outrages  against 
decency,  frauds,  assaults,  exhibitionism  and  the  like. 
To  be  sure  these  acts  are  absurd  and  infantile  and  not 
difficult  to  recognize  as  of  psychotic  origin. 


NEUROSYPHILIS  AND  THE  WAR  415 


Syphilis  may  bring  out  epilepsy  in  a  subject  having 
taint.     Case  from  Bonhoeffer,  1915. 


Case  I.*  A  man  of  35  in  the  Landwehr  acquired  syphilis 
some  time  in  the  summer  of  1914.  He  was  a  good  soldier, 
passed  through  several  clashes,  and  was  promoted  to  Unter- 
offizier. 

To  understand  what  followed  it  must  be  stated  that  he  had 
been  a  bed-wetter  to  n,  had  been  practically  a  teetotaler 
(Bonhoeffer's  point  is  perhaps  that  otherwise  epilepsy  might 
have  developed  sooner?),  and,  when  he  did  drink,  vomited 
almost  at  once,  and  had  amnesia  for  the  period  of  drunken- 
ness. His  father  had  been  somewhat  of  a  drinker.  His 
sister  had  suffered  from  convulsions  as  a  child. 

February,  1915,  the  Unteroffizier  lost  appetite,  got  head- 
aches, and  went  to  hospital  for  a  time.  Upon  getting  better, 
he  was  sent  on  service  to  Berlin.  In  a  Berlin  hotel  he  had 
his  first  convulsions  and  unconsciousness,  biting  his  tongue. 
He  was  confused  for  several  days,  and,  when  he  had  become 
clear,  had  a  pronounced  retrograde  amnesia  together  with  a 
tendency  to  fabricate  a  filling  for  the  lost  period. 

This  retrograde  amnesia  is  uncommon  in  epilepsy  and 
suggests  organic  disease.  No  sign  of  organic  disease  was 
found  on  neurological  examination.  The  patient  had  no 
signs  of  the  epileptic  make-up.  The  serum  W.  R.  was 
negative.  On  the  whole,  Bonhoeffer  regards  the  epilepsy  as 
"reactive  "  to  the  syphilis,  as  a  syphilogenic  epilepsy. 

As  to  the  amnesia,  it  is  of  interest  that  alcohol  should  long 
before  have  been  able  to  cause  amnesia  in  this  man  in  the 
same  way  as  does  now  the  syphilitic  epilepsy. 

I.  In  view  of  the  fact  that  this  Landwehr  man  appears  to 
have  acquired  syphilis  while  on  campaign,  what  is  the 
responsibility  of  the  government  for  treatment?  The 

*  Bonhoeffer.  Erfahrungen  iiber  Epilepsie  und  Ver- 
wandtes  im  Feldzuge.  Monatschr.  f.  Psychiat  u.  Neurol., 
Bd.  38,  H.  1-2,  1915. 


416  NEUROSYPHILIS  AND  THE  WAR 

Canadian  authorities,  as  stated  under  Case  E,  are  in 
doubt  whether  contraction  of  venereal  disease  con- 
stitutes negligence  on  the  part  of  the  soldier.  It  would 
appear  to  us  that  where  a  government  does  not  take 
suitable  steps  to  prevent  the  acquisition  of  syphilis  by 
the  soldiers,  the  government  must  assume  a  measure 
of  responsibility  for  the  syphilis  incurred.  The  govern- 
ment's responsibility  would  be  still  greater  in  equity, 
it  would  appear,  if  commercial  opportunities  for  the 
acquisition  of  syphilis  are  maintained  under  more  or 
less  close  government  supervision  or  (even  as  has  been 
claimed  for  certain  encampments  on  our  own  Mexican 
border)  if  shelter  for  illicit  sex  relations  is  afforded 
within  the  limits  of  a  military  camp.  In  a  certain  com- 
munity, "E,"  for  example,  it  is  claimed  by  Exner,*  the 
district  for  prostitutes  was  "situated  within  the  lines  of 
military  camps  and  protected  and  'regulated'  by  the 
military  authorities." 

But  even  if  the  government  has  no  legal  responsi- 
bility in  this  regard,  it  would  be  well  to  consider  the 
ultimate  results  of  the  syphilis  that  will  probably  be 
acquired  by  great  numbers  of  soldiers  under  campaign 
conditions.  Aside  from  the  ravages  of  syphilis  outside 
the  nervous  system,  it  is  well  known,  as  Weygandt 
intimates  for  German  conditions,  that  the  aftermath 
of  war  will  be  a  high  proportion  of  cases  of  neuro- 
syphilis. 

1  Weygandt  remarks  in  his  review  of  the  influence  of 
the  war  upon  psychiatry,  that  the  opportunity  for 
syphilitic  infection  in  the  campaign  is  considerable. 
In  the  war  of  1870,  the  conditions  in  this  regard  were 
extremely  unfavorable,  and  writing  in  1915,  Weygandt 
remarks  that  at  present  there  should  be  a  prophylaxis 
against  syphilitic  infection  by  the  soldiers,  which 
prophylaxis  should  be  the  most  energetic  possible. 
Continence  on  the  part  of  the  soldiers  and  the  isolation 
of  infected  women,  with  examination  by  specialists, 
have  been  advocated  by  Neisser  and  by  Mendel.  In 
the  '8o's  a  great  number  of  cases  of  locomotor  ataxia 
developed  in  Germany,  which  were  due  to  syphilis 
acquired  by  the  soldiers  and  officers  in  the  war  of  1870. 

'  Exner,  M.  J.,  Prostitution  in  its  relation  to  the  army  on. 
the  Mexican  Border,  Social  Hygiene,  Vol.  3,  2,  April,  1917. 


NEUROSYPHILIS  AND  THE  WAR  417 


Syphilis  in  a  psychopathic   subject.     Convulsions 
5  days  after  Dixmude.     Case  from  Bonhoeffer,  1913. 


Case  J.*  A  soldier  in  the  reserves,  23,  was,  subsequently 
to  his  being  brought  to  hospital,  described  by  his  wife  as  a 
rather  over-sensitive  fellow,  who  could  hardly  look  at  blood 
and  was  meticulous  about  the  household.  He  had  always 
been  subject  to  headaches,  especially  after  hard  work. 
However,  he  had  passed  through  his  military  training  well  in 
1910,  not  even  having  been  bestraft. 

He  began  service  in  October  and  fought  at  Dixmude  on 
the  iQth.  On  the  24th  in  the  trench  and  while  being  carried 
back,  he  had  several  spells  of  pallor,  falling  stiff,  and  then 
having  convulsions.  Brought  finally  to  the  Charite  in  Berlin, 
he  had  more  spells  of  sudden  pallor,  collapse  with  brief  con- 
vulsions, tossings  in  bed,  and  absences,  post-convulsive  head- 
aches, and  mild  bad  humor. 

There  were  numerous  attacks  several  days  apart  in  the 
first  seven  weeks.  The  patient  was  not  of  an  "epileptic  " 
disposition,  though  he  was  rather  readily  dissatisfied.  Head- 
aches also  occurred  without  relation  to  convulsions. 

The  serum  W.  R.  was  positive.  Treatment  by  mercurial 
inunctions.  No  further  convulsions.  Prognosis  as  to  the 
possibility  of  a  constitutional  epilepsy  unknown. 

*  Bonhoeffer,  loc.  cit. 


41 8  NEUROSYPHILIS  AND  THE  WAR 


SYPHILITIC  ROOT-SCIATICA  (lumbrosacral 
radiculitis)  in  a  fireworks  man  with  a  French  artil- 
lery regiment.  Case  presented  from  Dejerine's 
clinic  by  Long. 


Case  K.  No  direct  relation  of  this  example  of  root- 
sciatica  to  the  war  is  claimed  nor  was  there  a  question  of 
financial  reparation. 

There  was  no  prior  injury.  At  the  end  of  March,  1915, 
the  workman  was  taken  with  acute  pains  in  lumbar  region 
and  thighs,  and  with  urgent  but  retarded  micturition. 

Unfit  for  work,  he  remained,  however,  five  months  with  the 
regiment,  and  was  then  retired  for  two  months  to  a  hospital 
behind  the  lines.  He  reached  the  Salp6triere  October  12, 
1915,  with  "double  sciatica,  intractable." 

There  was  no  demonstrable  paralysis  but  the  legs  seemed 
to  have  "melted  away,"  fondu,  as  the  patient  said.  Pains 
were  spontaneously  felt  in  the  lumbar  plexus  and  sciatic 
nerve  regions,  not  passing,  however,  beyond  the  thighs. 
These  pains  were  more  intense  with  movements  of  legs;  but 
coughing  did  not  intensify  the  pains.  Neuralgic  points  could 
be  demonstrated  by  the  finger  in  lumbar  and  gluteal  regions 
and  above  and  below  the  iliac  crests  (corresponding  with 
rami  of  first  lumbar  nerves).  The  inguinal  region  was 
involved  and  the  painful  zone  reached  the  sciatic  notch  and 
the  upper  part  of  the  posterior  surface  of  the  thigh. 

The  sensory  disorder  had  another  distribution  objectively 
tested.  The  sacral  and  perineal  regions  were  free.  Anes- 
thesia of  inner  surfaces  of  thighs,  hypesthesia  of  the  anterior 
surfaces  of  thighs  and  lower  legs.  The  anesthesia  grew  more 
and  more  marked  lower  down  and  was  maximal  in  the  feet, 
which  were  practically  insensible  to  all  tests,  including  those 
for  bone  sensation.  There  was  a  longitudinal  strip  of  skin 
of  lower  leg  which  retained  sensation. 

Position  sense  of  toes,  except  great  toes,  was  poor.  There 
was  a  slight  ataxia  attributable  to  the  sensory  disorder  — 


NEUROSYPHILIS   AND  THE  WAR  419 

reflexes  of  upper  extremities,  abdominal,  and  cremasteric 
preserved,  knee-jerks,  Achilles  and  plantar  reactions  absent. 

The  vesical  sphincter  shortly  regained  its  function,  though 
its  disorder  had  been  an  initial  symptom. 

Pupils  normal. 

The  "sciatica"  here  affects  the  lumbosacral  plexus. 
Signs  of  disorder  at  one  time  or  other  affected  the  first  lumbar 
distribution  of  the  third  lumbar  and  first  and  second  sacral 
nerves. 

As  to  the  syphilitic  nature  of  this  affection,  there  had  been 
at  eighteen  (22  years  before)  a  colorless  small  induration  of 
the  penis,  lasting  about  three  weeks.  There  was  now  evident 
a  small  oval  pigmented  scar.  The  patient  had  married  at 
20  and  has  had  three  healthy  children. 

The  lumbar  puncture  fluid  yielded  pleocytosis  (120  per 
cmm.).  Mercurial  treatment  was  instituted. 

The  treatment  has  not  reduced  the  pains.  Long  thinks  it 
was  undertaken  too  long  (six  months)  after  onset.  The 
warning  for  early  diagnosis  is  manifest.  There  was  somehow 
a  delay  under  the  medical  conditions  of  the  army, 


420  NEUROSYPHILIS  AND  THE  WAR 


Can  the  "  lighting-up  "  of  NEUROSYPHILIS  IN 
CIVIL  LIFE  be  induced  by  the  domestic  stress  of 
war?  A  possible  example  from  Dr.  R.  Percy  Smith, 
London. 


Case  L.  A  German  Jew  in  London  passed  into  the 
PARETIC  form  of  NEUROSYPHILIS  shortly  after  the  outbreak 
of  war  under  conditions  suggesting  that  the  stress  of  emotions 
directly  or  indirectly  lighted  up  the  neural  process. 

The  man  was  a  bank-officer,  52  years  old,  and  married. 
He  had  lived  many  years  in  England  and  was  in  fact  a 
naturalized  citizen.  He  had  been  under  treatment  for 
syphilis  by  Sir  Jonathan  Hutchinson,  29  years  before,  namely, 
at  the  age  of  23.  Subsequently,  Sir  John  had  given  him  per- 
mission to  marry. 

It  proved  that  for  years  the  man  had  had  fixed  pupils, 
absent  knee-jerks,  and  a  perforated  ulcer  of  the  foot.  How- 
ever, there  had  been  no  other  mental  or  nervous  symptoms 
preventing  bank-officer's  work. 

At  the  outbreak  of  war  the  man  was  discharged  from  the 
bank.  He  grew  worried  and  sleepless.  He  began  to  charge 
himself  with  sex  irregularity.  He  went  down  to  the  city  and 
burned  trust  documents  belonging  to  others. 

From  worry  and  self-accusation  he  passed  into  depression 
and  agitation.  He  developed  a  belief  that  not  only  he  but 
also  his  German  wife  were  to  be  executed.  He  thought  he 
was  a  criminal  and  was  to  be  hanged. 

The  depression  then  altered  to  a  condition  of  hilarity  and 
loquacity. 

In  addition  to  the  fixed  pupils  and  absent  knee-jerks,  a 
speech  disorder  shortly  developed. 

The  patient  was  placed  under  care,  but  quickly  (a  few 
months?)  passed  into  an  advanced  stage  of  paretic  neuro- 
syphilis  and  died. 


NEUROSYPHILIS  AND  THE  WAR  42! 


SHELL-SHOCK  PSEUDOPARESIS  (non-syphili- 
tic). Recovery.  Case  from  Pitres  and  Marchand 
of  Bordeaux. 


Case  M.  June  19,  1915,  a  shell  exploded  some  distance 
from  Lieutenant  R.  He  remembers  the  gaseous  smell,  the 
bursting  of  several  shells  nearby  and  a  sensation  of  being 
lifted  into  the  air.  When  he  recovered  consciousness,  he  was 
in  hospital  at  Paris- Plage,  covered  with  bruises  and  scratches. 
They  told  him  he  had  been  delirious  and  had  vomited  and 
spat  blood. 

June  24,  his  wife  came  to  see  him,  but  this  visit  he  could 
not  remember.  Nor  could  his  wife  at  first  recognize  him,  he 
was  so  thin.  He  roused  a  few  moments  and  recognized  his 
wife,  but  relapsed  into  torpor  again.  Speech  was  difficult 
and  ideas  confused. 

A  few  days  later  he  was  able  to  rise;  but  his  mental  status 
grew  worse,  especially  as  to  speech  and  writing;  the  latter  quite 
illegible.  There  was  insomnia,  or,  if  he  slept,  war  dreams. 

August  7,  he  began  a  period  of  five  months'  convalescence 
passed  with  his  family,  depressed,  given  to  spells  of  weeping, 
confined  to  bed  or  couch,  unable  to  "find  words,"  conscious 
of  his  state  and  troubled  about  it,  speaking  of  nothing  but 
the  war,  and  afraid  to  go  out  for  fear  of  ambuscade.  There 
was  at  first  a  slight  lameness  of  the  right  leg.  Although  he 
could  walk,  he  felt  pain  in  the  knee  on  flexing  the  right  leg 
on  the  thigh.  He  walked  holding  this  leg  in  extension. 

On  going  back  to  the  colors,  he  was  immediately  evacuated 
to  the  Centre  Neurologique  at  Bordeaux,  January  20,  1916. 

Examination  found  a  bored,  impatient,  irritated  man,  vexed 
that  a  man  who  was  not  sick  should  be  sent  up  "comme  fou" 

Omitting  negative  details,  neurological  examination  showed 
slight  lameness  as  above,  body  stiff  and  movements  jerky; 
difficult,  unsteady  gait.  The  lieutenant  could  stand  for  some 
time  on  either  leg,  tongue  and  face  tremulous  during  speech. 
Limbs  moderately  tremulous,  especially  in  the  performance 
of  test  movements. 


422  NEUROSYPHILIS  AND  THE  WAR 

Knee-jerks  and  Achilles  jerks  absent.  Other  reflexes,  in- 
cluding pupillary,  normal.  Segmentary  hypalgesia  of  right 
leg,  especially  about  knee.  Tremulous  speech  and  writing. 
Patient  would  stop  short  in  speaking  for  lack  of  words. 

Malnutrition.  Appetite  good,  but  a  bursting  feeling  after 
meals. 

Skin  dry,  scaly  on  legs,  fissured  on  fingers. 

Serum  W.  R.  negative.     Fluid  not  examined. 

Mental  examination.  Conscious  and  complaining  of  his 
troubles,  Lieutenant  R.  claimed  persistently  that  he  was  not 
sick.  Memory  for  recent  events  was  in  general  poor.  Er- 
rands easily  forgotten.  Lost  in  the  street.  Complaint  of 
corpse  odors  round  him.  Everybody  is  looking  at  him  and 
making  fun  of  him.  He  was  apt  to  insult  bystanders.  He 
was  afraid  of  German  spies.  Things  in  shops  angered  him 
as  they  seemed  to  him  to  be  of  German  manufacture. 

There  were  frequent  periods  of  depression,  with  pallor  and 
no  spontaneous  speech  for  some  hours  to  a  half-day.  Head- 
aches coming  on  and  stopping  suddenly. 

As  to  diagnosis,  the  first  impression,  say  Pitres  and  Mar- 
chand,  was  that  of  general  paresis.  The  progress  of  symp- 
toms after  the  shock  was  consistent  with  this  diagnosis.  The 
mental  state  and  the  physical  findings  seemed  consistent, 
although  the  pupils  were  normal.  His  partial  insight  into 
his  symptoms  was  not  inconsistent  with  the  diagnosis.  He 
had  a  characteristic  self-confidence.  There  had  been  four 
stillbirths  (two  twins);  two  children  are  alive,  1 1  and  13. 
Typhoid  fever  at  30.  Syphilis  denied.  No  mental  disease 
in  the  family. 

The  patient  had  never  done  military  duty,  having  been 
invalided  for ' '  right  apex."  But  he  had  volunteered  and  been 
accepted  in  September,  1914. 

I.  Was  this  diagnosis,  general  paresis,  at  any  time  justified? 
The  spinal  fluid  should  of  course  have  been  examined. 
The  peculiar  lameness  of  the  right  leg  was  certainly 
not  characteristic  of  general  paresis,  and  was  perhaps 
hysterical.  (There  was  no  limitation  of  visual  fields  or 
any  other  definite  sign  of  hysteria.)  Presumably  some 
quality  of  speech  defect,  the  amnesia,  and  the  euphoria, 


NEUROSYPHILIS  AND  THE  WAR  423 

together  with  absent  knee-jerks,  led  to  the  diagnosis 
general  paresis.  By  the  2Oth  of  March,  1916,  the  knee- 
jerks  had  become  lively;  the  Achilles  jerks  normal.  At 
this  time  the  patient  had  gained  in  weight,  could  walk 
though  stiffly,  had  headache  (especially  right  frontal) 
and  a  feeling  of  lead  in  head,  less  tremor,  lack  of  desire 
to  undertake  anything.  He  still  wanted  to  go  back 
into  service.  He  still  saw  spies  about.  Dreams  ter- 
rible; devoured  by  spiders,  leggins  instruments  of 
torture.  Skin  still  atrophic.  June  4  there  was  no  more 
tremor  of  speech  or  face.  Symptoms  largely  disap- 
peared except  a  few  ideas  of  persecution.  Recovery 
October,  1916. 

How  was  Lieutenant  R.  cured?  Apparently  by  rest  in 
the  Centre  Neurologique.  Pitres  and  Marchand  do  not 
speak  of  the  subtle  relation  between  mental  state  and 
the  idea  of  non-return  to  military  service.  This  motive 
might  still  work  even  if  Lieutenant  R.  kept  protesting 
quite  sincerely  that  he  wanted  to  go  back  into  military 
service. 


424  NEUROSYPHILIS  AND  THE  WAR 


SHELL-SHOCK  PSEUDOTABES  (non-syphilitic, 
serum  W.  R.  positive).  Improvement.  Case  from 
Pitres  and  Marchand  of  Bordeaux. 


Case  N.  Innkeeper  B.,  36,  a  shell-shock  and  burial  victim 
June  20,  1915,  was  looked  on  by  a  number  of  physicians  as 
a  case  of  genuine  tabes. 

Even  eight  months  after  the  episode,  he  still  showed  (when 
observed  by  Pitres  and  Marchand,  February  3,  1916) 
absence  of  knee-jerks  and  Achilles  jerks,  a  slight  swaying  in 
the  Romberg  position,  pupils  sluggish  to  light,  incoordination, 
delayed  sensations.  There  was  also  a  history  of  pains  in  the 
legs,  compared  by  the  patient  to  those  of  sciatica.  These 
pains  came  in  crises,  the  longest  of  which  had  lasted  30  hours. 

It  seems  that  this  soldier's  troubles  began  the  day  after  his 
shock  with  a  feeling  of  swollen  feet  and  of  cotton  wool  under 
them.  He  stayed  on  service,  however,  walking  with  in- 
creasing difficulty. 

At  the  time  of  his  evacuation,  July  10,  he  could  walk  with 
great  difficulty.  "Strips  of  lead  were  between  his  legs."  He 
could  hardly  control  movements  in  the  dark,  or  descend 
stairs.  Often  his  legs  would  bend  under  him.  Vesical  func- 
tion sluggish. 

After  a  few  months  the  patient  could  walk  better.  On 
February,  1916,  he  walked  thrusting  his  legs  forward  trem- 
bling, and  dragging  toes  a  little.  He  could  not  support  him- 
self on  either  leg.  Jerkiness  and  incoordination  in  extension 
or  flexion  of  leg  on  thigh. 

The  muscular  weakness  was  decidedly  against  tabes  or  at 
all  events  a  pure  tabes.  The  incoordination  proved  to  be  due, 
not  to  loss  of  position  sense  (which  was  intact)  but  to  un- 
steady muscular  contractions.  Deep  sensibility  was  intact. 

There  were  no  mental  symptoms.  There  was  a  slight 
hesitation  in  speech  and  doubling  of  syllables,  but  nothing 
demonstrable  with  test  phrases. 

The  serum  W.  R.  was  positive.     Syphilis  denied. 


NEUROSYPHILIS  AND  THE  WAR  425 

I.  What  is  the  cause  of  these  phenomena?  Pitres  and 
Marchand  lean  to  the  hypothesis  of  slight  internal 
traumatism.  They  believe  that  there  is  either  (a)  slight 
internal  hemorrhage  in  the  nervous  system,  or  possibly 

(b)  what  they  call  "nerve  cell  contusion,"  or  perhaps 

(c)  caisson-disease-like  phenomena  from  aerial  decom- 
pression.    Some  authors  incriminate  (d)  the  gases.     It 
has  been   reported   by   certain   French   authors  that 
shortly  after  shell  shock  injury  or  burial  there  is  a 
pleocytosis  in  the  spinal  fluid  as  well  as  evidence  of 
hemorrhage.     The  pleocytosis  is  said  to  last  only  a  short 
time;    hence  when  patient  arrives  at  a  base  hospital 
lumbar  puncture  usually  discloses  nothing. 


Baalim  and  Ashtaroth 

Paradise  Lost,  Book  I,  line  422. 


VII.  SUMMARY  AND   KEY 

No  more  important  human  problem  now  exists  than 
syphilis.  Syphilis  of  the  nervous  system  or,  briefly,  neuro- 
syphilis  is  a  highly  important  fraction  of  the  total  problem. 
The  few  outstanding  dates  and  items  which  we  present  on  the 
following  page  give  but  a  faint  idea  of  the  amount  of  observa- 
tion and  thinking  which  the  medical  aspects  of  neurosyphilis 
alone  have  required.  The  present  work  deals  with  but  a  small 
fraction  of  the  results  of  this  work,  nor  can  we  more  than 
glance  at  the  scientific  history  of  syphilis  and  neurosyphilis 

—  a  history  that  would  form  an  epoch  in  itself. 

It  is  only  in  the  most  recent  years  that  syphilology  and 
the  narrower  science  of  neurosyphilology  have  threatened  to 
become  separate  disciplines  boasting  full  time  specialized 
workers.  Up  to  recent  years  the  contributions  to  the  theory 
of  syphilis  have  been  largely  by-products  of  work  in  larger 
sciences  and  arts.  Thus,  the  cellular  pathology  of  syphilis 
as  worked  out  by  Virchow  and  the  more  special  vascular 
features  as  worked  out  by  Heubner  were  incidental  in  the 
progress  of  pathological  anatomy  and  histology.  The  bold 
procedure  of  Quincke  in  proposing  lumbar  puncture  also  had 
its  more  general  ground  in  the  extension  of  clinical  medicine, 

—  an  interpretation  likewise  true  of  the  French  achievements 
in  the  cyto-diagnosis  and  chemical  diagnosis  of  the  lumbar 
puncture  fluids.     The  careful  histological  definitions  of  the 
Nissl-Alzheimer  group  were  incidental  to  the  application  of 
approved  and  classical  pathological  methods  to  neurological 
and  psychiatric  material. 

Again,  the  work  of  Schaudinn,  as  well  as  that  of  Metchnikoff 
and  Roux,  was  ingenious  work  with  the  methods  of  para- 
sitology  and  experimental  pathology.  The  great  work  of 
Schaudinn  in  establishing  the  constancy  of  the  spirocheta 
pallida  in  syphilis  may  be  said  to  have  started  syphilology 
as  something  approaching  a  special  discipline.  The  ideas 
of  one  of  the  greatest  of  immunologists,  Bordet,  were  almost 

427 


428 


SUMMARY  AND  KEY 


DATES,  NEUROSYPHILIS 


VIRCHOW 
HEUBNER 
QUINCKE 

RAVAUT,  SICARD, 
NAGEOTTI,  WIDAL 

WIDAL,  SICARD,  RAVAUT 

METCHNIKOFF  AND 
ROUX 

ALZHEIMER 


SCHAUDINN  AND 
HOFFMANN 

WASSERMANN,  NEISSER 
AND  BRUCK 

PLAUT 


EHRLICH 

SWIFT  AND  ELLIS 

NOGUCHI  AND  MOORE 
LANGE 


PATHOLOGY  1858 

ENDARTERITIS  1874 

LUMBAR  PUNCTURE    1891 

CYTODIAGNOSIS,  1901 

C.S.F. 

ALBUMIN,  C.S.F.  1903 

TRANSMISSION  TO       1903 
APES 

HISTOPATHOLOGY,       1904 
BRAIN  SYPHILIS 

SPIROCHETA  PAL-       1905 
LIDA 

SERUM  DIAGNOSIS       1906 

WASSERMANN  REAC-  1908 
TION,  C.S.F. 

SALVARSAN  1909 

SALVARSANIZED  1912 

SERUM 

SPIROCHETES,  BRAIN  1913 
TISSUE,  PARESIS 


GOLD  SOL  TEST 


1913 


CHART  28 


SUMMARY  AND  KEY  429 

immediately  applied  to  the  serum  diagnosis  of  syphilis  by 
Wassermann  and  the  further  application  of  this  method  to 
the  problems  of  neurosyphilis  was  almost  immediate,  with  the 
spirocheta  pallida  as  an  object  of  attack.  The  commanding 
intelligence  of  Ehrlich  could  at  once  seek  application  of  long 
incubated  ideas  of  chemotherapy  with  the  startling  outcome, 
salvarsan. 

The  history  of  syphilis  and  neurosyphilis  was  now  to  be 
thickly  sown  with  ideas  and  results  growing  from  the  achieve- 
ments of  Schaudinn  and  Ehrlich.  The  positive  reactions 
in  the  blood  and  spinal  fluid  in  the  most  striking  of  mental 
diseases,  general  paresis,  led  to  the  impression  that  general 
paresis  itself  might  at  last  be  proved  to  be  what  Moebius  had 
suspected,  namely,  100%  syphilitic.  We  know  how  difficult 
is  the  technical  proof  of  spirochetosis  in  the  brains  of  general 
paretics  both  post  mortem  and  ante  mortem,  but  no  one 
doubts  the  certainty  of  the  syphilitic  hypothesis  concerning 
the  origin  of  general  paresis. 

The  data  of  the  gold  sol  reaction  ultimately  obtained  from 
the  ideas  of  Thomas  Graham  concerning  colloids,  as  developed 
by  Szigmondi  and  effectively  applied  by  Lange,  have  broad- 
ened and  solidified  the  whole  plane  of  attack. 

The  ingenious  suggestions  of  Swift  and  Ellis  (salvarsanized 
serum)  and  the  notable  work  of  Noguchi  and  Moore  (spiro- 
chetosis in  paretic  brains)  indicate  to  us  as  Americans  what 
the  establishment  of  scientific  institutes  may  do  to  permit  the 
rapid  application  of  new  ideas  to  branches  of  inquiry  that  are 
opened  out.  Scientific  institutes  do  not  manufacture  a 
Virchow,  a  Metchnikoff,  a  Schaudinn,  a  Bordet  or  an  Ehrlich 
but  they  directly  permit  such  men  to  work  and  indirectly 
stimulate  the  development  of  more. 

The  series  of  137  cases  here  at  least  presented  does  not 
touch  systematically  the  problems  of  the  neuropathology  of 
syphilis,  which  would  themselves  require  a  textbook  of  respect- 
able size.  We  have,  however,  presented  in  Part  I,  cases  I  to  8, 
some  indication  of  the  protean  nature  of  the  material  and 
from  time  to  time  in  the  remainder  of  the  book  somewhat 
fuller  accounts  of  the  pathological  anatomy  and  histology 
have  been  presented  than  are  strictly  necessary  in  the.dem- 


430  SUMMARY  AND  KEY 

onstration  of  the  principles  of  modern  systematic  diagnosis 
and  treatment. 

Our  work  may  be  said  to  represent  psychopathic  hospital 
practice  as  available  to  us  in  our  official  capacities  at  the 
Psychopathic  Department  of  the  Boston  State  Hospital. 
A  word  is  necessary  concerning  the  nature  of  this  practice. 
The  dispensary  and  ward  practice  of  a  modern  state  psycho- 
pathic hospital,  such  as  the  Boston  institution  (founded  in 
1912)  and  the  Ann  Arbor  institution  (founded  in  1906),  is 
to  be  sharply  distinguished  from  asylum  practice.  Those 
who  have  not  followed  the  evolution  of  the  modern  psycho- 
pathic hospital  with  the  lowering  of  bars  to  the  admission  of 
patients  and  the  extension  of  its  benefits  to  a  group  of  sick 
persons  far  removed  from  the  medicolegal  concept  "  insan- 
ity "  may  not  soon  grasp  the  general  nature  of  psychopathic 
hospital  material.  Psychopathic  hospital  practice  stands, 
in  fact,  almost  midway  between  asylum  practice  in  the  classi- 
cal sense  and  private  practice.  This  has  come  about  through 
the  great  extension  of  the  so-called  voluntary  relation  under 
which  hundreds  of  patients  now  resort  to  the  beds  and  out- 
patient rooms  of  a  psychopathic  hospital,  who  would  formerly 
have  remained  untreated  or  inadequately  treated.  More- 
over, the  broadening  of  the  concept  of  mental  diseases  as  a 
whole  has  permitted  in  some  parts  of  the  world  the  establish- 
ment of  laws  under  which  psychopathic  and  psychotic  patients 
may  be  brought  to  psychopathic  hospitals  and  even  to  asylums 
under  the  easiest  possible  conditions  and  restrictions,  omitting 
court  procedure  altogether.  The  operation  of  the  voluntary 
and  temporary  care  provisions  of  law  has  accordingly  yielded 
us,  in  the  Boston  institution,  a  great  group  of  cases  formerly 
not  at  all  accessible  to  hospital  diagnosis  and  treatment. 
Needless  to  say,  as  always  under  such  conditions,  we  have 
been  able  to  show  not  merely  that  hospital  diagnosis  or  treat- 
ment is  of  importance  to  a  new  group  of  cases,  but  also  that 
home  treatment,  especially  home  treatment  under  super- 
vision, is  possible  and  even  ideal  for  a  large  group  of  cases 
about  which  utter  darkness  or  profound  misgivings  ruled  in 
the  not  very  distant  past. 

Accordingly,  we  are  fain  to  insist  that  our  material  is  of 


SUMMARY  AND  KEY  431 

importance  in  new  programs  of  community  organization  for 
the  stamping  out  of  disease.  The  work  in  psychopathic 
hospitals  upon  neurosyphilis  in  particular  is  essentially  a 
part  of  the  public  health  program,  although  our  special  work 
will  not  soon  be  taken  over  by  the  public  health  officers, 
so  complicated  are  the  ramifications  of  medical  and  social 
diagnosis  and  treatment  in  the  neurosyphilis  group. 

We  have  tried  in  Part  IV  (medicolegal  and  social  cases) 
to  give  a  few  examples  to  illustrate  the  part  played  by  neuro- 
syphilis in  society;  but  we  regard  this  part  of  our  work  as  the 
least  satisfactory  and  the  least  representative  in  the  total 
work.  Our  colleagues  in  social  service,  in  mental  hygiene, 
in  psychopathology  and  in  criminology  will  easily  in  the  next 
few  years  provide  a  far  more  adequate  basis  for  a  full  account 
of  the  public  and  social  aspects  of  neurosyphilis.  One  point 
we  should  emphasize  here.  The  psychopathic  hospital  worker, 
whether  physician  or  social  worker,  must  shortly  decide 
upon  and  consolidate  a  program  with  relation  to  the  families 
of  neurosyphilitics. 

The  syphilographers  of  the  dermatological  and  special 
syphilis  clinics  have  their  identical  problems  with  the  families 
of  syphilitics;  but  the  dispensaries  for  mental  cases  and  in 
particular  the  psychopathic  hospital  and  asylum  out-patient 
departments  tap  another  reservoir  of  syphilitic  families  at  a 
stage  when  the  memory  of  the  initial  horrors  of  syphilitic 
infection  is  dimmed  or  erased.  Any  program  for  the  diagnosis 
and  treatment  of  syphilis  of  the  innocent  must  take  into 
account  not  only  the  skin,  syphilis,  and  internal  medicine 
clinics  but  also  the  clinics  for  mental  and  nervous  diseases 
wherein  neurosyphilitics  are  not  infrequent.  Whether  the 
ultimate  percentage  will  stand  at  10,  15  or  20%  for  the  neuro- 
syphilitics in  mental  clinics,  is  of  no  importance  to  the  prin- 
ciple. There  are  enough  neurosyphilitics  having  economical 
importance  and  humanly  precious  families  to  warrant  definite 
steps. 

The  Massachusetts  Commission  for  Mental  Diseases  has 
in  the  last  few  years  employed  the  services  of  two  medical 
workers  whose  time  has  been  largely  devoted  to  the  applica- 
tions of  our  recent  knowledge  in  neurosyphilis  and  has  gone 


432  SUMMARY  AND  KEY 

so  far  as  to  establish  a  neurosyphilis  ward  in  one  of  the  district 
state  institutions  (Summer  Street,  Worcester,  under  the 
Graf  ton  Hospital  Board).  Special  social  workers  in  the 
field  of  neurosyphilis  have  also  been  available  from  time  to 
time.  These  social  workers  are  enabled  with  the  support 
of  the  medical  profession  to  do  a  great  deal  of  good,  for  ex- 
ample, with  the  slogan  THE  CHILD  OF  A  PARETIC  is  THE 
CHILD  OF  A  SYPHILITIC. 

The  nature  of  the  intake  of  patients  into  psychopathic 
hospital  wards  and  out-patient  clinics  is  such  that  great 
numbers  of  non-mental  syphilitics  arrive  for  diagnosis  and 
possible  treatment.  Moreover,  the  existence  of  syphilis  in 
non-suspects  is  a  fact  picked  up  by  the  way  in  routine  Was- 
sermann  serum  diagnosis. 

The  mental  clinic  in  the  modern  sense  with  the  medico- 
legal  bars  lowered  or  well  nigh  removed,  turns  rapidly  into 
a  clinic  for  neurological  cases  as  well.  The  German  models 
for  mental  and  nerve  clinics  are  rapidly  being  imitated. 
The  result  of  this  administrative  novelty  in  our  hospital 
procedure  has  incidentally  yielded  us  many  representative 
cases  of  entirely  non-psychotic  and  even  non-psychopathic 
neurosyphilis.  Our  impression  grows  and  deepens  that  the 
neurosyphilitic  is  seldom  merely  a  spinal  syphilitic.  The 
neurosyphilitic  is  nearly  always  the  victim  not  merely  of 
spinal  disease  but  also  of  intracranial  disease.  Per  contra, 
the  victim  of  intracranial  neurosyphilis  is  almost  always 
more  or  less  importantly  affected  by  spinal  neurosyphilis. 

The  net  result  of  the  modern  work  on  neurosyphilis  has 
been  to  bring  the  neurologist  and  the  psychiatrist  together 
upon  one  platform  in  diagnosis  and  more  and  more  upon  one 
platform  in  treatment.  But  aside  from  the  clinical  evidence 
that  the  neurosyphilitic  is  apt  to  be  a  victim  of  both  brain 
syphilis  and  cord  syphilis,  the  autopsy  evidence  is  stronger 
still.  Even  the  victim  of  tabetic  neurosyphilis  ("  tabes 
dorsalis  ")  himself  is  rarely  found  at  autopsy  without  more 
or  less  evidence  of  significant  encephalic  disease  of  a  chronic 
inflammatory  or  degenerative  nature.  Aside  from  tabes 
dorsalis  and  Erb's  paraplegia,  the  rule  is  almost  universal 
that  neurosyphilis  is  a  matter  of  the  entire  nervous  system. 


SUMMARY  AND  KEY  433 

In  view  of  the  generalization  of  neurosyphilitic  process, 
one  might  question  the  advantage  of  any  topical  grouping  of 
neurosyphilitic  disease.  Practically  speaking,  however,  as  we 
have  shown  in  Chart  5,  it  seems  advisable  to  separate  the 
neurosyphilitic  diseases  into  six  roughly  distinguishable  groups. 
First,  there  is  the  great  group  that  we  have  chosen  to  term 
diffuse  neurosyphilis,  including  many  of  the  cases  of  so-called 
cerebral  or  cerebrospinal  syphilis  of  the  neurological  clinics 
and  the  group  of  cases  that  have  been  treated  in  private 
practice  by  internists  and  neurologists  without  recourse  to 
institutions.  These  cases  have  lived  at  home  and  have  not 
been  socially  hard  to  manage  until  the  late  phases  of  their 
disease  when  the  victims,  if  poor,  are  sent  to  almshouses  and 
infirmaries  under  municipal  or  state  care.  These  are  the  cases 
which  have  been  in  the  past  regarded  as  most  amenable  to 
the  classical  iodid  and  mercurial  treatment.  Indeed  there 
is  record  of  numerous  therapeutic  successes  in  the  group. 

Whereas  the  lesions  in  diffuse  neurosyphilis  are  chiefly 
chronic  inflammatory  and  degenerative  changes  of  a  diffuse 
nature  (with  vascular  changes  incidental  or  subordinate  to 
the  inflammation  and  the  degeneration),  there  is  an  important 
and  large  group  of  cases  that  we  have  termed  vascular  neuro- 
syphilis in  which  the  factors  of  inflammation  and  degeneration 
are  subordinate  to  vascular  insults.  These  are  cases  of  syphi- 
litic arteriosclerosis  and  the  best  examples  are  victims  of 
cerebral  thrombosis.  The  clinical  symptoms  of  the  immediate 
attacks  (of  apoplectiform,  epileptiform  or  other  acute  nature) 
are  not  in  themselves  distinguishable  from  the  immediate 
effects  of  non-syphilitic  vascular  disease;  nevertheless  the 
establishment  of  their  syphilitic  etiology  is  of  the  utmost 
importance  on  account  of  the  possibilities  of  treatment  of 
the  underlying  syphilis.  For,  as  the  neuropathologist  must 
always  insist,  the  immediate  effects  of  vascular  insults  whether 
syphilitic  or  non-syphilitic  are  much  more  extensive  than  the 
ultimate  paralytic  or  residual  irritative  effects;  and  by  con- 
sequence a  greater  optimism  is  justifiable  in  the  confronting  of 
these  cases  than  the  nihilistic  observer  is  likely  to  entertain. 

Physicians  dealing  with  chronic  disease  in  general  are  apt 
to  be  somewhat  nihilistic,  but  this  nihilism  is  increased  a 


434  SUMMARY  AND   KEY 

hundred  fold  in  disease  of  the  nervous  system.  How  im- 
portant then  is  any  work  which  shall  demonstrate  partial 
or  even  complete  recovery  from  serious  looking  apoplectic 
and  other  seizures,  besides  all  of  which  the  point  of  syphilitic 
treatment  naturally  lies  in  the  prevention  of  future  insults 
of  the  same  sort.  Therapeutic  experience  in  this  vascular 
group  has  almost  as  good  a  toll  of  successes  as  in  the  diffuse 
neurosyphilis  group  above  mentioned,  that  is  to  say,  the 
modern  systematic  treatment  and  even  the  old  pre-salvarsan 
treatments  have  succeeded  fairly  well  in  removing  the  products 
of  inflammation  from  the  membranes  of  the  nervous  system 
and  in  abolishing  vascular  disease. 

The  old  principle  that  the  dead  neurone  in  the  central 
nervous  system  cannot  be  regenerated  remains  a  perfectly 
firm  principle ;  but  there  are  any  number  of  neurones  and  even 
neurone  systems  that  are  not  essential  to  life  or  to  the  pursuit 
of  happiness.  We  accordingly  have  just  as  good  a  theoreti- 
cal therapeutic  outlook  in  many  instances  of  chronic  neuro- 
syphilis as  we  have  in  chronic  diseases  of  many  other  organs. 
Add  to  this  the  fact  that  a  great  number  of  the  most  sharply 
defined  and  grave  symptoms  are  probably  not  due  to  de- 
struction of  neurones  but  to  irritation  and  functional  dis- 
ability of  neurones,  and  the  conclusion  is  compelled  that,  as 
hinted  above,  an  entirely  unjustifiable  pessimism  and  nihilism 
have  prevailed  in  some  quarters.  Of  course,  the  recoil  from 
such  pessimism  with  the  onset  of  salvarsan  treatment  led 
various  enthusiasts  to  an  undue  optimism. 

Another  great  group  distinguished  by  the  existence  of 
spinal  cord  disease  is  the  group  we  have  termed  tabetic 
neurosyphilis,  which  group  contains  the  classical  tabes  dor- 
salis  or  locomotor  ataxia  and  its  congeners. 

The  question  of  therapeutic  optimism  comes  up  most 
forcibly  in  the  field  of  tabes.  It  is  hard,  however,  at  this 
time  to  give  a  proper  and  scientifically  founded  estimate  of  the 
therapeutic  outcome  in  tabetic  neurosyphilis  with  modern 
methods.  So  much  can  be  said :  namely,  that  the  alleviation 
of  pain  and  the  palliation  of  other  symptoms  can  be  success- 
fully claimed  as  a  result  of  the  renewed  interest  in  the  treat- 
ment of  this  affection.  What  was  said  above  concerning  the 


SUMMARY  AND   KEY  435 

finality  of  the  death  process  in  a  dead  neurone  is  very  strik- 
ingly true,  of  course,  of  some  of  the  neurones  of  the  posterior 
columns  in  tabes  dorsalis.  Still  only  portions  of  these  neu- 
rones (namely,  those  which  run  an  intradural  course)  are 
strikingly  altered  in  a  great  many  cases.  Now  and  again 
one  is  greatly  astonished  to  observe  the  restoration  of  the 
lost  knee-jerk  in  cases  of  neurosyphilis  (see  for  instance  the 
case  of  Alice  Morton  (i),  with  discussion).  In  short,  the  re- 
lation of  several  tabetic  symptoms  to  irritative  conditions 
and  functional  disability  of  neurones  may  be  considered 
established.  Naturally,  moreover,  if  therapy  can  stop  the 
upward  course  of  the  affection  as  it  passes  from  lower  to 
higher  nerve  roots  (according  to  reasonably  well-established 
ideas  of  the  genesis  and  progress  of  this  affection),  we  are 
entitled  to  a  further  degree  of  optimism. 

The  question  of  therapeutic  optimism  versus  pessimism  is 
forced  upon  attention  in  the  fourth  great  group  of  neurosyph- 
ilitic  diseases  which  we  have  chosen  to  distinguish,  namely,  the 
group  of  paretic  neurosyphilis  including  the  disease  formerly 
known  as  general  paresis,  paralytic  dementia,  softening  of 
the  brain  and  the  like. 

Of  course,  no  one  can  gainsay  there  is  a  group  of  cases 
having  in  the  natural  course  of  events  a  prognosis  of  fatality 
within  a  term  of  years,  say  three  to  five  years,  and  we  have 
cases  in  our  series  which  go  to  show  that  even  with  the  modern 
intensive  treatment  the  characteristic  down-grade  sympto- 
matic progress  and  ultimate  fatality  occur.  Still,  we  have 
other  cases  diagnostically  on  all  fours  with  the  fatal  cases 
that  have  seemed  to  get  either  entirely  well  with  the  labora- 
tory tests  returning  to  normal  and  without  further  mental 
symptoms,  or  else  lose  mental  symptoms  on  the  one  hand  or 
laboratory  signs  on  the  other.  We  should  strongly  object 
to  any  account  of  paretic  neurosyphilis  which  should  insist 
that  its  necessary  outcome  is  fatality  within  a  term  of  years. 
Of  course,  viewing  our  knowledge  of  the  affection  in  the  past, 
we  should  be  compelled  to  object  to  the  generalization  "  par- 
esis fatal"  on  the  evidences  of  the  universally  recognized  re- 
missions. If  nature  can  stop  a  paretic  process,  why  cannot 
man  do  as  much?  Can  it  be  alleged  that  our  own  apparent 


436  SUMMARY  AND   KEY 

therapeutic  successes  and  those  of  others  are  merely  curious 
examples  of  coincidences,  namely,  that  remissions  have  chosen 
to  occur  precisely  when  therapy  was  systematically  applied? 
The  percentage  of  therapeutic  successes  with  modern  intensive 
treatment,  wherever  it  may  ultimately  stand,  is  already  too 
high  for  this  hypothesis  of  fortuitous  remissions.* 

Moreover,  we  believe  that  the  details  of  the  clinical  progress 
of  some  of  the  reported  cases  are  convincing  on  this  point. 
What,  however,  is  the  distinguishing  feature  of  paretic  neuro- 
syphilis?  It  is  in  one  sense  a  particular  kind  of  diffuse  neuro- 
syphilis.  The  tissues  are  apt  to  show  not  only  encephalic  but 
also  spinal  changes.  There  is  apt  to  be  a  more  or  less  well- 
defined  meningitis,  but  the  characteristic  feature,  without 
which  the  diagnosis  of  paretic  neurosyphilis  would  hardly 
be  rendered,  is  the  existence  of  disease  of  the  cerebral  cortex. 
This  disease  is  parenchymatous  in  the  sense  of  showing  nerve 
cell  destruction.  There  is  also  an  interstitial  reaction  in  the 
shape  of  a  neuroglia  overgrowth,  but  the  striking  and  pathog- 
nomonic  feature  is  the  infiltration  of  the  sheaths  of  the 
small  vessels  in  the  cortex,  giving  evidence  of  an  inflammation 
very  intimately  affecting  the  cellular  mechanisms  of  the 
nervous  system.  It  is  striking  how  often  a  smaller  or  larger 
share  of  the  cells  found  in  the  vessel  sheaths  are  plasma  cells. 
It  does  not  appear,  however,  that  the  diagnosis  of  paretic 
neurosyphilis  as  against  diffuse  non-paretic  neurosyphilis  can 
be  made  in  the  stained  sections  with  complete  safety  on  the 
basis  of  plasmocytosis  in  the  former  and  lymphocytosis  in 
the  latter.  Whatever  the  results  of  careful  histological  differ- 
entiation by  future  neuropathologists  may  yield,  it  is  at  all 
events  true  that  we  cannot  yet  make  an  important  differen- 
tiation clinically  on  the  basis  of  the  differential  count  of 
plasma  cells  and  lymphocytes  in  the  puncture  fluids.  How- 

*  We  have  recently  reviewed  the  outcome  in  300  untreated 
cases  of  paretic  neurosyphilis  (Psychopathic  Hospital  ma- 
terial, strictly  comparable  with  treated  cases)  finding  but  5 
now  capable  of  self-support  and  10  more  in  normal-looking 
remission.  This  percentage  is  far  lower  than  that  in  treated 
cases  (at  present,  July,  1917,  50  in  200  capable  of  self- 
support). 


SUMMARY  AND  KEY  437 

ever  this  may  be,  there  is  an  important  distinction  between 
diffuse  neurosyphilis  of  the  non-paretic  type  and  paretic  neuro- 
syphilis  in  that  paretic  neurosyphilis  rarely  if  ever  fails  to 
show  important  degrees  of  intracortical  perivascular  inflam- 
mation with  larger  or  smaller  numbers  of  plasma  cells. 

What  has  the  therapeutist  to  face  in  this  matter?  The 
answer,  as  elsewhere,  depends  somewhat  upon  what  the  future 
may  decide  as  to  the  habitat  and  toxic  or  antitoxic  activities 
of  the  spirocheta  pallida.  The  early  claims  that  the  spi- 
rocheta  pallida  was  extravascular  and  lay  for  the  most  part 
in  the  parenchyma  and  not  in  the  vessel  sheaths  were  perhaps 
overbold,  since  other  workers  have  found  the  spirochete  in 
the  vessel  sheaths  also  (Mott). 

Aside  from  the  spirochete  and  its  accessibility  to  spiro- 
chetocidal  drugs,  there  seems  to  be  no  reason  for  supposing 
that  the  perivascular  sheaths  cannot  be  cleansed  of  their 
inflammatory  contents.  There  is,  again,  no  reason  why  the 
phagocytic  cells  should  not  continue  to  perform  their  scavenger 
function  until  such  time  as  the  degenerative  process  in  the 
parenchyma  (a  process  not  necessarily  progressive  in  the  ab- 
sence of  the  spirochete  or  its  products)  ceases.  There  is  every 
reason  to  suppose  that  a  great  many  of  the  clinical  phenomena 
are  not  necessarily  due  to  permanent  destruction  of  neurones 
and  neuronic  organs  (dendrites,  axis-cylinders,  nets  and  the 
like)  but  are  due  to  various  microphysical  conditions  of 
pressure,  intoxication  and  the  like. 

The  inflammatory  conditions  in  the  spinal  cord  of  poliomye- 
litis, which  conditions  are  precisely  as  striking  as  those  of  the 
paretic  cortex,  are  beyond  a  question  cleared  away  in  the 
progress  of  the  affection.  Reference  to  the  paradigm  case  (l) 
will  show  the  type  of  our  argument.  There  is  no  manner  of 
doubt  that  in  this  paradigm  case  almost  every  portion  of  the 
nervous  system  had  been  sometime  swept  by  spirochetosis 
and  many  of  its  small  vessel  sheaths  stuffed  with  chronic 
inflammatory  products.  As  for  paretic  neurosyphilis  itself, 
a  great  many  of  its  most  striking  clinical  phenomena,  such  as 
loss  of  memory  and  disorientation,  as  well  as  great  degrees  of 
apparent  dementia,  are  found  virtually  as  often  in  cases  with 
very  slight  anatomical  changes  as  in  cases  with  marked  cortical 


438  SUMMARY  AND   KEY 

devastation.     The  inference  is  plain,  that  these  phenomena 
are  to  a  degree  functional  rather  than  structural. 

In  brief,  we  conclude  not  only  from  therapeutic  experience 
but  also  on  a  priori  grounds  that  the  histological  conditions  in 
paretic  neurosyphilis  are  not  entirely  hopeless,  and  certainly 
not  more  hopeless  than  conditions  in  many  chronic  diseases 
outside  the  nervous  system.  Accordingly,  we  plead  for  a  tem- 
perate optimism  as  to  therapeutic  results  in  general  paresis. 

A  fifth  group  of  neurosyphilitic  cases  bulking  rather  largely 
in  textbooks  of  pathology  is  the  group  of  the  gummata. 
For  a  variety  of  reasons  (therapeutic  and  otherwise)  the 
actual  number  of  gummata  of  the  nervous  system  available 
for  clinical  or  even  for  anatomical  study  is  much  smaller  than 
the  books  might  lead  one  to  infer. 

The  sixth  and  last  of  the  main  groups  of  neurosyphilitic 
diseases  is  that  of  the  juvenile  forms,  among  which  we  find  not 
only  diffuse  forms  without  a  special  and  well-defined  course, 
but  also  characteristic  examples  of  paretic  and  tabetic  neuro- 
syphilis. The  distinction  of  a  juvenile  or  congenital  group 
of  neurosyphilitics  is,  on  theoretical  grounds,  perhaps  hardly 
defensible.  On  practical  grounds,  however,  the  juvenile 
neurosyphilitics  do  form  a  group  having  special  relations  to 
feeblemindedness,  epilepsy  and  the  like. 

We  must  be  clearly  understood  as  to  the  rough,  six-unit 
classification  just  given.  It  is  practical  merely.  For  com- 
parison we  have  given  in  other  charts  more  expanded  lists 
of  the  diagnostic  entities  in  neurosyphilis  among  which  that 
of  Head  and  Fearnsides  is  of  special  interest,  see  Chart  2, 
page  21. 

We  shall  now  proceed  to  a  brief  analysis  of  the  findings  in 
our  chosen  series  of  137  cases.  We  shall  not  reproduce  the  case 
headings  of  these  cases,  but  expand  their  statements  where 
necessary  and  tie  them  together  so  far  as  possible  into  a 
reasonable  and  systematic  statement  of  the  situation  in  neuro- 
syphilis. The  footnotes  will  contain  references  to  other  cases 
in  which  identical  points  are  illustrated  as  in  the  leading 
cases.  The  leading  cases  will  in  all  instances  be  placed  first 
in  the  footnotes. 


SUMMARY  AND  KEY  439 

The  paradigm  *  shows  meningeal,  vascular  and  paren- 
chymatous  lesions  and  thus  illustrates  our  definition  of  the 
term  DIFFUSE  which  means  precisely  meningeal,  vascular  and 
parenchymatous.  The  meningeal  lesions  gave  rise  to  two 
prominent  sets  of  lesions,  first,  the  marked  tabetic  lesions  of 
the  spinal  cord  (due  to  the  spinal  root  neuritis  incidental  to 
the  spinal  meningeal  inflammation),  secondly,  the  character- 
istic asymmetrical  and  focal  atrophy  of  cranial  nerves  inci- 
dental to  a  now  largely  extinct  meningeal  process  at  the  base 
of  the  brain.  The  vascular  lesions  are  responsible  for  another 
important  and  characteristic  factor  in  the  case,  namely,  the 
bilateral  pyramidal  tract  sclerosis ;  the  bilateral  cysts  of  soften- 
ing of  the  corpora  striata  are  characteristic  effects  of  old 
syphilitic  cerebral  thromboses.  The  parenchymatous  disease 
in  our  paradigm  is  everywhere  obvious,  less  so  perhaps  in 
the  cortex  itself  than  elsewhere,  although  here  also  evident 
in  the  shape  of  lesions  suggesting  an  early  phase  of  tissue 
atrophy. 

The  paradigm  is  of  interest  in  demonstrating  what  in  broad 
lines  must  be  taken  as  an  ascending  disease  proceeding  not 
only  from  spinal  cord  to  encephalon  but  also  traceable  as 
proceeding  from  lower  parts  of  the  spinal  cord  to  upper  parts 
thereof  and  from  the  lower  encephalon  to  the  higher  structures 
of  the  cerebral  cortex  itself. 

The  paradigm  insistently  calls  attention  to  the  advantage 
of  persistent  therapy  not  only  in  its  display  of  remarkable 
successive  recoveries  from  permanent  looking  symptoms  but 
also  histologically  from  the  remnants  of  inflammatory  process 
to  be  found  in  an  otherwise  almost  wholly  dismantled  nervous 
system  with  extinct  lesions. 

TABETIC  NEUROSYPHILIS  f  ("tabes  dorsalis"),  of  course, 
often  proceeds  to  death  without  special  complications  of  syphi- 
litic nature.  We  have  chosen  a  case,  however,  to  demonstrate 
a  terminal  complication  with  vascular  insult.  Incidentally  the 
case  shows  another  complication  inasmuch  as  the  cause  of 
death  was  rupture  of  aortic  aneurysm.  It  is  important  to 
bear  in  mind  these  complications  in  tabes  dorsalis  which  go 

*  Alice  Morton  (i).  f  Francis  Garfield  (2). 


440  SUMMARY  AND   KEY 

to  prove  that  the  spirochetosis  of  tabetic  neurosyphilis  is  not 
limited  to  the  region  of  the  spinal  roots  or  to  the  spinal  region 
in  general.  Tabetic  neurosyphilis  is  apt  to  be  only  a  part  of  a 
total  picture  of  neurosyphilis  just  as  neurosyphilis  itself  is 
only  a  part  of  the  general  syphilitic  process. 

Our  case  of  PARETIC  NEUROSYPHILIS  *  ("  general  paresis  ") 
is  a  characteristic  one  in  duration  (three  years  and  three 
months).  The  aortic  sclerosis  almost  constantly  found  in 
neurosyphilis  and  especially  in  paretic  neurosyphilis  is  here 
also  shown.  The  spinal  cord  showed  lesions  which  are  also 
almost  always  found  in  paretic  neurosyphilis.  The  character- 
istic frontal  emphasis  of  the  atrophic  and  indurative  lesions  is 
shown.  There  is  also  a  display  of  gross  changes  in  the  pia 
mater.  The  characteristic  so-called  granular  ependymitis  or 
sanding  of  the  ventricular  surface  is  shown.  The  case  is  dis- 
tinguishable from  the  paradigm  in  not  showing  the  effects  of 
vascular  insults  in  the  shape  of  cysts  of  softening.  The  cere- 
bellar  sclerosis  of  the  case  is  fairly  characteristic  of  paretic 
cases.  There  is  even  a  suggestion  of  atrophy  in  the  temporal 
region  suggesting  the  so-called  Lissauer's  paresis.  Clinically 
the  case  belongs  in  the  classical  grandiose  group  of  paretics 
("  O.  K.  No.  I  superfine  "). 

VASCULAR  NEUROSYPHILIS  f  is  illustrated  in  a  fourth  autop- 
sied  case.  It  may  be  noted  that  the  pia  mater  in  this  case  is 
practically  normal.  The  tissues  outside  the  area  of  softening 
due  to  the  syphilitic  thrombosis  of  nutrient  vessels  are  practi- 
cally normal.  The  case  was  one  of  almost  complete  sensory 
aphasia  with  word-deafness.  The  clinical  picture  is  accord- 
ingly quite  distinct  from  those  of  the  paradigm  (i)  and  of  the 
case  of  general  paresis  (3)  just  discussed. 

JUVENILE  PARESIS  J  is  illustrated  by  a  case  with  exceedingly 
extensive  lesions,  largely  meningeal  and  parenchymatous. 
The  cerebral  lesions  are  atypical  since  in  places  they  suggest 
the  tuberous  sclerosis  of  Bourneville.  The  brain  atrophy  is 
extreme  (965  grams)  and  it  is  possible  that  this  apparent 
brain  atrophy  was  in  part  hypoplasia,  since  the  spirochetosis 

*  John  Dixon  (3).  f  James  Pierce  (4). 

|  John  Lawrence  (5)  m 


SUMMARY  AND  KEY  44! 

of  this  case  was  doubtless  congenital.  However,  clinically 
the  patient  was  fairly  normal  up  to  the  age  of  1 8. 

A  case  of  so-called  SYPHILITIC  EXTRAOCULAR  PALSY  *  de- 
monstrates a  characteristic  meningeal  process  more  extensive 
than  the  clinical  symptoms  would  have  indicated.  In  fact, 
focal  clinical  nerve  palsies  are  as  a  rule,  if  not  constantly, 
partial  phenomena  of  a  far  more  extensive  process  of  neuro- 
syphilis.  They  are  far  more  limited  clinically  than  anatom- 
ically and  histologically.  It  seems  at  first  sight  improper 
to  term  them  cases  of  diffuse  neurosyphilis  in  view  of  their 
clinical  focality,  yet  they  are  best  described  as  partial  cases  of 
diffuse  neurosyphilis. 

A  case  of  GuMMAf  of  the  left  HEMISPHERE  is  presented 
which  appears  to  have  led  to  death  in  about  four  years  from  on- 
set. This  case,  like  many  others,  is  not  an  example  of  purely 
focalized  syphilitic  process  inasmuch  as  cysts  of  softening 
indicating  slight  vascular  insults  are  present  elsewhere  (pons). 
There  is  also  a  degree  of  leptomeningitis,  particularly  basal. 

Our  discussion  of  the  nature  and  forms  of  neurosyphilis 
is  completed  by  a  rare  case  probably  belonging  in  the  so- 
called  cervical  hypertrophic  meningitis  of  Charcot  but  actually 
due  to  a  GUMMA  OF  THE  SPINAL  MENINGES.J  The  import- 
ance of  therapeutic  optimism  is  emphasized  in  this  case  as  in 
the  paradigm.  Theoretically  the  meningeal  inflammation  of 
neurosyphilis  ought  to  be  almost  entirely  if  not  entirely 
removed  by  therapy,  and  these  two  cases,  like  several  others 
in  the  series,  seem  to  illustrate  this  possibility. 

Neurosyphilis  sometimes  receives  the  clinical  diagnosis  neu- 
rasthenia simply  through  omission  to  apply  proved  diagnostic 
methods.  An  instance  is  given  in  which  the  PARETIC  form 
of  NEUROSYPHILIS  ("  general  paresis  ")  received  the  diagnosis 
neurasthenia  §  for  a  period  of  five  years,  at  any  time  during 
which  period  it  would  doubtless  have  been  possible  to  render 
the  correct  diagnosis  and  apply  treatment. 

*  Flora  Black  (6).  §  Greeley  Harrison  (9).  Also 

.  ,  Albert  Robinson  (45), 

f  Mrs.  Lecompte  (7).  Alice  Caperson  (46), 

J  John  Wyman  (8).  Abel  Bachmann  (74). 


442  SUMMARY   AND   KEY 

Neurosyphilis  may  imitate  not  only  the  psychoneuroses 
but  also  the  psychoses  themselves.  We  present  a  case  of  an 
architect,  which  looked  almost  precisely  like  manic-depressive 
psychosis  *  and  had  a  history  of  attacks,  but  in  which  the 
positive  serum  W.  R.  led  (in  accordance  with  hospital  rules) 
to  an  examination  of  the  spinal  fluid.  The  spinal  fluid  tests 
proved  the  case  to  be  one  of  PARETIC  NEUROSYPHILIS. 

However,  a  positive  serum  W.  R.,  even  when  associated 
with  mental  symptoms,  and  when  those  mental  symptoms 
include  grandiosity,  does  not  prove  the  existence  of  neuro- 
syphilis  either  in  its  paretic  or  non-paretic  form.  Our  in- 
stance seems  to  be  one  of  MANIC-DEPRESSIVE  PSYCHOSIS,  f 
The  spinal  fluid  tests  were  entirely  negative.  The  course  of 
the  disease  was  also  that  of  manic-depressive  psychosis.  In 
the  absence  of  positive  spinal  fluid  tests,  the  diagnosis  neuro- 
syphilis  was  excluded. 

Neurosyphilis  and  even  PARETIC  NEUROSYPHILIS  may 
result  in  symptoms  that  would  ordinarily  lead  to  the  diag- 
nosis dementia  praecox.\ 

It  is  important  not  to  rule  out  neurosyphilis  on  the  ground 
of  a  negative  serum  W.  R.  The  fluid  W.  R.  may  turn  out 
positive.  We  present  a  case  (of  a  salesman)!  in  which  the 
serum  W.  R.  was  repeatedly  negative  (even  salvarsan  did 
not  act  provocatively)  yet  the  spinal  fluid  W.  R.  proved 
positive.  The  case  was  clinically  one  of  classical  PARETIC 
NEUROSYPHILIS  ("general  paresis").  It  is  a  good  rule  to  pro- 
ceed to  lumbar  puncture,  even  when  the  serum  W.  R.  is  nega- 
tive, if  there  are  suspicious  symptoms  (e.g.,  speech  defect  and 


*  Lyman  Agnew  (10).  Also        J  Henry  Philipps  (12).  Also 
Ethel  Hunter  (47),  Bridget  Curley  (59), 

Bessie  Vogel  (52),  Margaret  O'Brien  (68), 

Isaac  Thompson  (83),  Annie  Martin  (117). 

Juliette  Lachine  (n). 

§  William  Twist  (13).   Also 

f  Juliette  Lachine  ( 1 1).  Also  Lester  Crane  (20), 

Lyman  Agnew  (10),  Thomas  Donovan  (23). 

Ethel  Hunter  (47), 
Bessie  Vogel  (52), 
Isaac  Thompson  (83). 


SUMMARY  AND   KEY  443 

memory  impairment,  grandiosity)  or  signs  (e.g.,  marked  reflex 
disorder,  especially  pupillary  disorder). 

DIFFUSE  NEUROSYPHILIS  was  above  denned  as  "meningo- 
vasculoparenchy matous. ' '  This  disease  is  typically  associated 
with  six  positive  tests  (positive  serum  W.  R.,  positive  fluid 
W.  R.,  pleocytosis,  gold  sol  reaction,  positive  globulin  re- 
action and  excess  albumin).  One  or  more  and  frequently 
several  of  these  six  tests  are  likely  to  run  mild  in  diffuse 
neurosyphilis;  that  is  to  say,  these  tests  are  apt  to  run  milder 
than  the  identical  tests  in  paretic  neurosyphilis  ("general 
paresis").  The  clinical  course  of  the  diffuse,  and  especially 
the  meningovascular  cases,  is  likely  to  be  protracted.  The 
prognosis  as  to  life  is  good,  barring  fatal  vascular  insults. 
The  illustrative  case  *  was  a  case  with  slow  course.  There 
was  a  series  of  attacks  followed  by  a  paralytic  stroke,  a  find- 
ing highly  typical  of  the  diffuse  form  of  neurosyphilis.  The 
spinal  fluid  reactions  were  mild,  suitable  to  the  general  prin- 
ciple above  stated. 

These  tests  are  likely  to  run  stronger,  as  above  stated, 
in  paretic  neurosyphilis  ("  general  paresis  "),  than  in  the 
diffuse  form.  In  particular,  the  gold  sol  reaction  is  likely 
to  be  shown  in  what  is  termed  "  paretic  "  form  rather  than 
in  what  is  termed  "  syphilitic  "  form.  The  clinical  course  of 
PARETIC  NEUROSYPHILIS  is  likely  to  be  brief.  A  character- 
istic case  f  with  very  heavy  globulin  and  albumin  tests  is 
presented. 

TABO-PARETIC  NEUROSYPHILIS  {  ("  taboparesis  ")  is  clin- 
ically a  combination  of  the  symptoms  of  tabetic  ("tabes 
dorsalis ")  and  those  of  paretic  neurosyphilis  ("  general 
paresis ").  First  comes  the  tabes  dorsalis  lasting  often 
for  many  years.  Afterward  follows  a  characteristic  general 
paresis.  The  ultimate  paretic  picture  is  likely  to  retain, 
however,  various  characteristics  of  tabes.  The  laboratory 
tests  in  the  paretic  phase  of  taboparesis  are  characteristic 

*  John  Jackson  (14).   Also  f  Pietro  Martiro  (15).     Also 
Martha  Bartlett  (21),  Meyer  Levenson  (22), 

Paolo  Marini  (28),  Achilles  Akropovlos  (50). 

Margaret  O'Brien  (68).  J  Joseph  Sullivan  (16). 


444  SUMMARY  AND  KEY 

of  general  paresis  and  not  of  tabes  dorsalis.  The  prognosis 
after  the  paretic  phase  has  arrived  is  that  of  general  paresis. 

The  diagnosis  of  the  neurosyphilitic  forms  would  be  easy 
if  these  principles  were  always  carried  out  to  the  letter. 
The  important  fact  is  as  follows:  diffuse  (that  is,  meningo- 
vasculoparenchymatous  neurosyphilis)  may  look  like  paretic 
neurosyphilis  ("  general  paresis  ")  *  at  certain  periods  of  the 
clinical  and  laboratory  examination.  This  fact  is  of  obvious 
importance.  The  general  prognosis  of  diffuse  neurosyphilis 
is  regarded  as  good  quoad  vitam.  The  general  prognosis  of 
paresis  is  bad.  If,  however,  the  differential  diagnosis  cannot 
be  rendered  at  particular  phases  of  a  given  case,  then  no  safe 
prognosis  can  be  offered  in  the  individual  case.  In  particu- 
lar no  prognosis  affecting  the  administration  or  non-adminis- 
tration of  modern  systematic  treatment  can  or  should  be 
offered  in  these  doubtful  phases. 

It  is  not  always  safe  to  exclude  neurosyphilis  even  when  the 
fluid  W.  R.  is  negative,  f  Particularly  in  vascular  neurosyphi- 
lis the  fluid  W.  R.  and  even  all  the  other  laboratory  signs 
in  the  spinal  fluid  may  sometimes  be  negative.  A  positive 
serum  W.  R.  yields  the  correct  pointer  to  diagnosis.  Of 
course,  also  in  many  cases  of  vascular  neurosyphilis  one  or 
more  of  the  laboratory  signs  may  be  suggestive  even  when 
the  fluid  W.  R.  is  negative.  Theoretically  there  may  be 
cases  in  which  all  the  six  tests  are  negative  and  yet  the  diag- 
nosis neurosyphilis  be  the  correct  one. 

A  clinically  important  sign  in  neurosyphilis  is  the  so-called 
seizures.  These  occur  both  in  DIFFUSE  NON-PARETIC  NEURO- 
SYPHILIS J  and  in  PARETIC  NEUROSYPHILIS.§ 

*  Gregorian  Petrof ski  (17).  Also  J  Agnes  O' Neil  (19).  Also 

Richard  Lawlor  (25),  Michael  O'Donnell  (24). 

John  Bennett  (34),  John  Edwards  (104). 

Julius  Kantor  (54),  Arthur  Bright  (121). 

Albert  Forest  (112). 

§  Lester  Crane  (20).    Also 
f  Frederick  Wescott  ( 1 8).    Also       Greeley  Harrison  (9). 

Martha  Bartlett  (21),  David  Borofski  (49). 

James  Burns  (56),  David  Collins  (61). 

Victor  Friedburg  (108). 


SUMMARY  AND  KEY  445 

Aphasia  is  likewise  a  symptom  in  both  these  forms  of 
neurosyphilis,  namely,  in  the  DIFFUSE  non-paretic  *  and  in 
the  PARETIC  form.f 

The  literature  contains  reference  not  only  to  seizures  and 
aphasia  as  characteristically  paretic  but  also  to  remissions. 
Remissions  like  seizures  and  aphasia  are  found  in  both  the 
PARETIC  J  and  NON-PARETIC  forms  of  neurosyphilis.  §  They 
have  important  bearings  on  prognosis  in  all  forms  of  neuro- 
syphilis and  are  of  especial  significance  in  the  evaluation  of 
treatment.  (Remissions  coincident  with  apparent  cure.) 

So  far  we  have  been  dealing  with  cases  of  neurosyphilis 
in  which  there  was  no  doubt  of  the  existence  of  mental 
symptoms.  There  are  cases,  however,  in  which  although  the 
laboratory  signs  of  neurosyphilis  exist,  proving  beyond  doubt 
the  existence  of  a  chronic  inflammatory  reaction  and  allied 
pathological  conditions  in  the  cerebrospinal  axis,  there  are  no 
mental  symptoms  of  neurosyphilis.  We  have  called  some  of 
these  cases  PARESIS  SINE  PARESI  ||  and  present  examples. 

To  illustrate  complications  we  give  a  case  of  PARETIC  NEU- 
ROSYPHILIS with  autopsy  in  which  there  were  ante  mortem 
signs  of  HERPES  ZOSTER  f  or,  at  all  events,  of  a  skin  eruption 
limited  to  the  area  of  a  thoracic  nerve. 

A  case  of  GUMMA  of  the  brain  **  in  which  decompression 
was  warranted  and  performed  is  presented.  The  fluid  W.  R., 
as  in  many  such  cases,  was  negative;  serum  positive. 

A  case  of  CRANIAL  NEUROSYPHILIS  (extraocular  palsy  ff 
without  mental  symptoms)  showed  a  positive  Wassermann 
serum  test  and  a  negative  spinal  fluid. 

*  Martha  Bartlett  (21).  Also      \\  Richard  Lawlor  (25).     Also 
Agnes  O'Neil  (19),  Bessie  Vogel  (52), 

Vivian  Walker  (87).  -  (88). 

t  Meyer  Levenson  (22).  Also     f  John  Morrill  (26). 

Albert  Forest  (112).  **  Davki  Tannenbaum  (27). 

$  Thomas  Donovan  (23).  Also       Also  Mrs.  LeCompte  (7), 

William  Twist  (13),  Annie  Rivers  (109). 

Bessie  Vogel  (52),  ^  paolo  Marini  (28).    Also 

David  Collins  (61).  plora  Black  (6). 

§  Michael  O'Donnell  (24).  Also 

Alice  Morton  (i). 


446  SUMMARY  AND   KEY 

The  laboratory  reactions  in  TABETIC  NEUROSYPHILIS  * 
("  tabes  dorsalis  ")  run  somewhat  like  those  of  diffuse  non- 
paretic  neurosyphilis  and  are  accordingly  milder  than  those 
of  paretic  neurosyphilis.  The  fluid  W.  R.  and  the  gold  sol 
reaction  in  particular  are  apt  to  run  mild.  The  clinical  course 
of  tabes  dorsalis  is  well  known  to  be  protracted  and  the  prog- 
nosis quoad  vitam  is  good  except  that  we  must  alway  bear  in 
mind  the  possibility  of  vascular  insults  and  complications 
of  a  syphilitic  origin  in  the  rest  of  the  body. 

It  is  important  to  remember  that  TABETIC  NEUROSYPHILIS 
is  often  quite  atypical  f  clinically  and  may  even  show  no 
single  symptom  warranting  the  old  clinical  name  locomotor 
ataxia. 

There  are  even  cases  in  which  the  name  tabes  dorsalis  is 
not  warranted  in  view  of  the  fact  that  the  lesions  are  not 
low  in  the  cord  but  are  higher  up  (TABES  CERVICALIS|). 

A  rare  form  of  neurosyphilis  is  ERB'S  SYPHILITIC  SPASTIC 
PARAPLEGIA  §  against  which  one  needs  to  consider  a  number 
of  non-syphilitic  spinal  cord  diseases.  Our  case  showed  a 
weakly  positive  serum  W.  R.,  a  negative  fluid  W.  R.,  and  the 
other  tests  of  the  spinal  fluid  were  moderately  positive. 

SYPHILITIC  MUSCULAR  ATROPHY  ||  is  classified  by  Head  and 
Fearnsides  both  in  their  meningovascular  group  and  in  their 
group  of  the  so-called  syphilis  centralis.  Our  case  affecting 
in  large  part  the  small  muscles  of  the  hands  in  a  teamster, 
may  be  due  either  to  spinal  parenchyma!  lesions  or  to  root 
neuritis  or  to  both. 

It  is  a  little  extraordinary  and  very  important  that  the 
laboratory  signs  are  apt  to  be  positive  even  in  the  SECONDARY 
period  of  SYPHILIS.  Perhaps  a  third  of  all  cases  of  syphilis  in 
the  secondaries  would,  if  tested,  yield  positives  precisely 
like  those  of  full-blown  paretic  or  diffuse  neurosyphilis. 

*  Mario  Sanzi  (29).    Also      f  Stephen  Green  (30).  Also 
Stephen  Green  (30),  Paul  Halleck  (31), 

Paul  Halleck  (31).  Henri  Lepere  (105), 

Ivan  Rokicki  (in). 
J  Paul  Halleck  (31). 
§  Margaret  Neal  (32). 
||  Joseph  Graham  (33). 


SUMMARY  AND   KEY  447 

Strangely  enough,  these  signs  may  occur  without  clinical 
symptoms.  The  illustrative  case,*  a  mechanic,  yielded  vari- 
ous mental  symptoms.  The  cases  of  secondary  syphilis  with 
laboratory  signs  of  neurosyphilis  but  without  clinical  symp- 
toms are  of  the  greatest  theoretical  importance  in  relation 
to  the  problem  above  mentioned  of  paresis  sine  paresi.  It 
may  well  be  inquired  whether  in  some  instances  the  neuro- 
syphilis of  the  secondaries  does  not  persist  until  the  ex- 
hibition of  mental  or  physical  symptoms  of  neurosyphilis 
years  later.  It  must  be  remembered  that  this  conception  is 
hardly  more  than  a  hypothesis  at  the  present  time.  That 
such  signs  of  chronic  inflammation  could  exist  without 
symptoms  is  not  so  surprising  when  one  thinks  of  the  startling 
immediate  improvement  seen  after  treatment  or  even  in 
remissions  without  treatment.  One  is  reminded  of  the  crisis 
in  pneumonia  wherein  clinical  improvement  takes  place  en- 
tirely independent  of  the  mechanical  conditions  in  the  lung 
which  just  after  the  crisis  remain  as  suppurative  as  before. 

The  diagnosis  of  JUVENILE  NEUROSYPHILIS  is  made  upon 
the  same  lines  as  that  of  neurosyphilis  in  the  adult.  We  pre- 
sent two  cases,  one  with  optic  atrophyf  and  the  other  with  signs 
of  congenital  syphilis  antedating  the  symptoms  of  paresis.! 

Congenital  syphilis  is  also  apparently  capable  of  producing 
a  simple  form  of  FEEBLEMINDEDNESS,  §  that  is  to  say,  a  form 
of  disease  non-paretic,  non-tabetic,  and  without  special  tend- 
ency to  vascular  insults. 

We  present  a  case  of  JUVENILE  TABETIC  NEUROSYPHILIS 
("  juvenile  tabes  ").||  The  tests  were  all  positive. 

The  line  of  separation  between  typical  and  atypical  cases 
of  neurosyphilis  is  vague  and  indistinct  and  some  of  the 

*  John  Bennett  (34).     Also  %  Theresa  Mullen  (36).  Also 
Alice  Caperson  (46),  John  Lawrence  (5), 

Florence  Fitzgerald  (81),          John  Friedreich  (77), 
Vivian  Walker  (87),  Gridley  Ringer  (78), 

Arthur  Bright  (121).  James  Arnold  (80). 

t  Mary  Coughlin  (35).  §  Isaac  Goldstein  (37). 

II  Archibald  Sherry  (38). 


448  SUMMARY  AND  KEY 

cases  classified  by  us  amongst  puzzles  perhaps  belong  under 
systematic  diagnosis  and  vice  versa.  The  section  on  PUZ- 
ZLES AND  ERRORS  in  the  diagnosis  of  neurosyphilis  is 
introduced  by  six  cases  of  error  in  the  diagnosis  of  the  paretic 
form  of  neurosyphilis.*  These  errors  were  made  known  by 
autopsy.  Aside  from  the  sixth  case,  whose  etiology  must  re- 
main in  doubt  and  which  was  a  unique  case  of  PERIVASCULAR 
GLIOSIS,  there  is  ground  for  the  belief  that  the  other  five  cases 
in  this  Danvers  Hospital  study  of  diagnostic  errors  were  per- 
haps actually  syphilitic  though  not  of  the  paretic  form  of 
neurosyphilis.  At  all  events,  the  brain  tissues  in  these  cases 
failed  to  show  the  plasma  cell  deposits  which  are  characteristic 
in  the  sheaths  of  the  intracortical  vessels  in  paretic  neuro- 
syphilis. 

A  case  illustrates  the  complication  of  TABES  by  arterio- 
sclerotic  symptoms,  in  which  case  the  arteriosclerosis  may 
naturally  have  been  of  syphilitic  origin.  Two  cases  especially 
illustrate  the  possibility  of  confusing  the  ataxia  of  general 
paresis  with  CEREBELLAR  ATAXIA.  These  cases  showed 
lesions  of  the  cerebellar  structures,  notably  of  the  dentate 
nucleus.  No  one  can  read  these  cases  or  any  of  the  autop- 
sied  cases  in  our  series,  without  perceiving  how  fundamental 
and  even  critical  is  the  demand  for  autopsies  in  fatal  cases 
of  neurosyphilis.  The  practitioner  who  can  secure  an  autopsy 
in  a  fatal  case  of  neurosyphilis  and  have  the  tissues  worked 
up  by  approved  neuropathological  methods  is  almost  bound 
to  add  his  bit  to  neurological  theory.  Even  cases  of  classical 
tabes  dorsalis  are  often  signally  important  to  the  theorist  on 
account  of  the  relations  of  the  neural  to  the  non-neural  com- 
plications. 

We  then  proceed  to  a  group  of  cases  without  special  order 
in  which  a  variety  of  diagnostic  questions  arose. 

A  case  of  questionable  neurosyphilis  in  the  secondary  stage 
of  syphilis  brings  up  the  problems  of  syphilitic  neurasthenia.^ 

*  Caroline  Davis  (39).  Elizabeth  Brown  (42). 

H.  F.  (40).  Robert  Allen  (43). 

Samuel  North  (41).  John  Hughes  (44). 

f  Albert  Robinson  (45).     Also 
Greeley  Harrison  (9). 


SUMMARY  AND   KEY  449 

Syphilis  may  act  as  agent  provocateur  of  HYSTERIA  as 
Charcot  insisted.* 

A  case  illustrative  of  difficulties  in  diagnosis  between  neuro- 
syphilis  and  manic-depressive  psychosis  follows.f 

A  case  for  diagnosis  is  given  which  shows  that  errors  in 
the  diagnosis  of  neurosyphilis  are  entirely  possible  even  when 
abundant  clinical  and  laboratory  data  are  available.  A  case 
with  a  weakly  positive  Wassermann  reaction  in  the  spinal 
fluid  finally  turned  out  to  be  one  of  BRAIN  TUMOR.J 

Some  questions  as  to  the  diagnosis  of  NEUROSYPHILIS  versus 
Idiopathic  Epilepsy  are  brought  up  by  a"  case  in  which  phe- 
nomena of  paresis  seemed  to  have  occurred  very  early,  about 
two  years  after  the  initial  syphilitic  infection.  § 

A  case  of  PARETIC  NEUROSYPHILIS  is  offered  in  which 
hemiplegia  and  hemitremor  strongly  suggested  vascular  lesions; 
but  the  autopsy  showed  no  coarse  lesions  and  merely  con- 
firmed the  diagnosis  paresis  microscopically.  || 

An  autopsied  case  of  PARETIC  NEUROSYPHILIS  is  given,  in 
which  the  pupils  persisted  in  reacting  normally.  Herpes 
zoster-like  lesions  in  life  yielded  no  special  signs  at  autopsy 
(all  root-ganglia  looked  alike  above  and  below  zone  of 
"shingles.")! 

An  example  of  NEUROSYPHILIS,  probably  PARETIC,  yielded 
symptoms  highly  suggestive -'of  manic-depressive  psychosis*4 
An  interesting  feature  in  this  case  was  the  birth  of  a  healthy 
child  nine  months  after  the  onset  of  the  psychotic  attack. 

An  example  of  exophthalmic  goitre  ft  following  the  ac- 
quisition of  SYPHILIS  showed  at  autopsy  a  heavy  scarring  of 
the  optic  thalamus  and  unilaterally  atrophic  process  in  the 
cerebral  cortex. 

*  Alice  Caperson  (46).  Also       §  David  Borosfski  (49).  Also 
Florence^Fitzgerald  (81).  Lester  Crane  (20). 

f  Ethel  Hunter  (47).  Also  \\  Achilles  Akropovlos  (50). 

Lyman  Agnew  (10),  If  Daniel  Wheelwright  (51). 

Bessie  Vogel  (52),  **^Bessie  Vogel  (52).    Also 

Juliette  Lachine  (n).  Lyman  Agnew  (10), 

$  Milton  Safsky  (48).    Also  Juliette  Lachine  (il), 

Daniel  Falvey  (55).  Ethel  Hunter  (47). 

ft  Carrie  Pearson  (53). 


450  SUMMARY   AND    KEY 

We  come  to  some  questions  concerning  the  Argyll-Robert- 
son pupil.  It  is  agreed  on  all  hands  that  the  Argyll- Robert- 
son pupil  is  characteristic  of  the  paretic  and  tabetic  forms, 
but  the  sign  occurs  also  in  other  neurosyphilitic  conditions;* 
in  fact  the  sign  does  not  necessarily  indicate  neurosyphilis 
as  an  instance  of  PINEAL  TUMOR  demonstrates.! 

The  question  raised  above  as  to  the  possibility  that  neuro- 
syphilis may  exist  in  the  absence  of  positive  findings  in  the 
spinal  fluid  is  illustrated  in  a  man,  a  mechanic,  who  claimed 
syphilitic  infection  and  showed  an  Argyll-Robertson  pupil 
on  one  side.J  The  serum  W.  R.  was  positive;  the  fluid  tests 
were  negative. 

An  extraordinary  case  is  given  in  some  detail  in  which 
NEUROSYPHILIS  in  the  form  termed  DISSEMINATED  ENCEPH- 
ALITIS §  proved  fatal  within  seven  months  of  the  initial  in- 
fection. 

We  have  frequently  mentioned  the  classical  assumption 
that  paretic  neurosyphilis  ("  general  paresis  ")  is  a  fatal  disease. 
Some  have  suggested  that  there  is  another  form  clinically 
almost  identical  with  general  paresis  except  that  it  pursues 
a  long  course  and  the  suggestion  has  been  made  that  these 
cases  be  termed  pseudoparesis .  We  are  of  the  opinion  that 
this  term  should  be  dropped  and  advocate  the  use  of  the 
word  pseudoparesis  only  for  non-syphilitic  disease  looking 
like  paresis,  such  as  alcoholic  pseudoparesis  and  the  like. 

The  question  whether  there  is  a  form  of  mental  disease 
SYPHILITIC  PARANOIAC  is  raised  by  a  case  with  auditory  hal- 
lucinations, ideas  of  persecution  and  attacks  of  excitement. 
The  diagnosis  of  alcoholic  hallucinosis  was  actually  made  al- 
though there  is  no  proof  that  the  patient  ever  drank  alcohol. 

Alcohol    may   cause    symptoms    identical   with    those  of 

*  Julius  Kantor  (54).     Cf.  J  James  Burns  (56).     Also 
James  Burns  (56).  Frederick  Wescott  (18), 

Henri  Lepere  (105).  Martha  Bartlett  (21), 

Frederick  Stone  (106).  Victor  Friedburg  (108). 

t  Daniel  Falvey  (55).     Cf.    §  John  Summers  (57). 
Francis  Murphy  (60).         ||  peter  Burkhardt  (58). 

^  Bridget  Curley  (59). 


SUMMARY   AND   KEY  45! 

paretic  neurosyphilis,  including  seizures,  Argyll-Robertson 
pupils,  speech  defect  and  mental  symptoms.  The  differen- 
tation  is  readily  made  by  the  negative  laboratory  findings. 
An  illustration  is  given  in  our  case  of  the  alcoholic  teamster. 
Cases  such  as  this  bear  the  name  ALCOHOLIC  PSEUDO- 
PARESIS.* 

However,  when  the  clinical  picture  is  the  same  as  in  the 
case  of  our  teamster,  the  alcohol  may  only  be  a  complicating 
factor  in  neurosyphilis,  as  shown  by  our  next  case  of  the 
alcoholic  steamfitter  who  in  fact  was  shown  to  have  NEURO- 

SYPHILIS.f 

Sometimes  cases  of  apparently  frank  alcoholism,  even  with 
apparently  characteristic  delirium  tremens  and  neuritis,  prove 
to  be  essentially  neurosyphilitic.J  On  the  other  hand,  true 
combinations  of  ALCOHOLISM  and  NEUROSYPHILIS  occur  which 
it  would  be  proper  to  classify  under  either  heading  and  in  which 
therapy  must  take  serious  account  of  both  conditions.  § 

As  above  stated,  we  elect  to  use  the  term  pseudoparesis  only 
for  non-syphilitic  cases.  There  are  other  forms  of  pseudo- 
paresis  than  alcoholic  pseudoparesis.  The  question  of  Dia- 
betic Pseudoparesis  is  raised  by  an  exceedingly  complicated 
case  of  which  our  best  interpretation  is  that  the  patient,  a 
proved  syphilitic  (with  syphilitic  osteomyelitis  (?)),  a  huge 
doorkeeper,  was  perhaps  suffering  from  an  old  SYPHILITIC 
scarring  of  the  PITUITARY  body.||  Neither  this  case  nor  a 
second  case,  one  of  PARETIC  NEUROSYPHILIS  with  glycosuria 
is  actually  entitled  to  the  diagnosis  diabetic  pseudoparesis. 
The  second  case  of  paretic  neurosyphilis  with  glycosuria 
brings  up  some  unanswerable  questions  as  to  the  pancreatic 
or  basal  meningitic  or  other  origin  for  the  glycosuria.^ 

Isolated  symptoms  are  often  presented  by  neurosyphilitics 
(e.g.,  hemianopsia)  ;**  but  we  tend  to  regard  these  cases  as  due 
to  focal  lesions  that  are  merely  part  and  parcel  of  DIFFUSE 
LESIONS. 

*  Francis  Murphy  (60).         §  Albert  Fielding  (63). 
f  David  Collins  (61).  ||  Calvin  Hall  (64). 

J  Joseph  Buck  (62).  K  Donald  Barrie  (65). 

**  Lawrence  Washington  (66) 


452  SUMMARY  AND  KEY 

A  neurosyphilitic  case  (a  steward)  with  the  rather  unusual 
complication  (for  our  northern  region)  of  severe  MALARIA 
producing  cerebral  thrombosis  is  reported.* 

The  diagnosis  Dementia  Praecox^  was  actually  made  in 
the  case  of  a  young  school  teacher  in  whom  the  laboratory 
findings  proved  conclusively  that  the  condition  was  one  of 
NEUROSYPHILIS.  The  gold  sol  reaction  in  this  case  was  mild. 
The  chief  lesion  at  autopsy  was  a  fresh  looking,  gelatinous 
pial  exudate  over  the  spinal  cord  which  turned  out  to  contain 
an  almost  pure  display  of  very  numerous  plasma  cells. 

The  question  of  LUES  MALIGNA  |  is  brought  up  in  a  rectifier 
of  spirits  in  whom  the  characteristic  tremendous  destruction 
of  tissue,  toxemia  and  failure  to  react  to  antisyphilitic  treat- 
ment were  illustrated.  Moreover,  this  case  had  a  trauma 
(cautery)  to  the  tonsil,  as  in  other  cases  of  lues  maligna. 

A  case  somewhat  suggestive  of  brain  tumor,  of  neuro- 
syphilis  and  of  multiple  sclerosis^  turned  out  to  be  MULTIPLE 
SCLEROSIS  (the  fluid  showed  a  pleocytosis  and  a  moderate 
amount  of  globulin  with  a  paretic  type  of  gold  sol  reaction). 

As  a  foil  to  this  case  that  we  regard  as  multiple  sclerosis, 
we  present  a  second  case  with  nystagmus,  optic  atrophy  and 
spasticity  in  which  the  suspicion  of  multiple  sclerosis  might 
well  be  raised  but  which  the  tests  demonstrated  to  be 

NEUROSYPHILITIC.  1 1 

An  even  stranger  imitation  of  well-defined  non-syphilitic 
entities  was  presented  by  a  case  apparently  of  Huntington's 
chorea^l  (except  for  absence  of  the  hereditary  taint)  which 
case,  however,  proved  to  the  surprise  of  all  diagnosticians  to 
be  one  of  NEUROSYPHILIS. 

Frequent  errors  of  diagnosis  must  occur  in  the  field  of  the 
senile  psychoses.  We  present  a  case  that  would  at  first 
blush  warrant  the  diagnosis  of  senile  arterio  sclerotic  psychosis** 

*  Joseph  Temple  (67).  t  Frank  Mason  (69). 

t  Margaret  O'Brien  (68).  Also  §  Annie  Kelly  (70). 
Henry  Phillips  (12).  James  Lauder  (71). 

Bridget  Curley  (59).  ||  James  Lauder  (     } 

Annie  Martin  (117). 

Tf  Margaret  Green  (72). 

**  Marcus  Chatterton  (73). 


SUMMARY  AND  KEY  453 

in  a  sea  captain  of  75  years  (wife  dead  15  years  before  of 
general  paresis)  who  turned  out  to  be  a  characteristic  case 
from  the  laboratory  standpoint  of  NEUROSYPHILIS. 

The  Protean  nature  of  the  symptomatology  of  neurosyphilis 
is  sufficiently  established.  Still,  a  case  that  might  fit  into  text- 
books concerning  DISSOCIATION  OF  PERSONALITY  *  is  certainly 
a  clinical  oddity,  as  illustrated  by  a  fugacious  musician. 

A  case  with  strong  suspicions  of  neurosyphilis  of  tabetic 
type  turned  out  to  be  more  probably  one  of  neural  com- 
plications in  PERNICIOUS  ANEMIA.! 

NEUROSYPHILIS  IN  JUVENILES  presents  puzzling  conditions. 

One  case  was  marked  clinically  by  attacks  of  excitement. % 
It  is  impossible  to  place  this  case  among  the  main  groups  of 
juvenile  neurosyphilis. 

Another  case  of  FEEBLEMINDEDNESS,  §  also  NEUROSYPHI- 
LITIC  in  origin,  presented  physical  symptoms  and  labora- 
tory signs  of  paretic  neurosyphilis;  yet  this  case  had  been 
considered  one  of  simple  feeblemindedness. 

A  case  apparently  of  JUVENILE  PARETIC  NEUROSYPHILIS  in 
a  15  year  old  boy  presented  the  rather  unusual  complication 
of  shocks  with  quadriplegia,|  |  a  vascular  complication  not 
usually  expected  in  the  paretic  type  of  neurosyphilis  in  adults. 

Epileptic  phenomena^  are  rare  as  the  effect  of  JUVENILE 
NEUROSYPHILIS,  but  occur  as  demonstrated  in  a  case  which 
slipshod  methods  of  diagnosis  might  well  have  regarded  as 
one  of  idiopathic  epilepsy. 

A  case  of  JUVENILE  PARETIC  NEUROSYPHILIS  with  the 
complication  of  ADDISON'S  DISEASE**  is  given  (autopsy 
confirmation). 

The  puzzle  in  diagnosis  offered  by  syphilis  in  the  secondary 
stage  f  f  is  illustrated  by  a  case  which  showed  the  characteristic 
NEUROSYPHILITIC  complications  of  the  SECONDARY  STAGE  of 

*  Abel  Bachman  (74).  **  James  Arnold  (80). 

f  Mrs.  Brown  (75).  ft  Florence  Fitzgerald  (81).  Also 

%  James  Seabrook  (76).  John  Bennett  (34), 

5  Tohn  Friedreichf??)   Cf  Alice  Caperson  (46), 

§  Isaac  Goldstein(37J  Vivian  Walker  (87) 

||  Gridley  Ringer  (78).  Arthur  B"ght  (l2l)" 

1[  John  Doran  (79). 


454  SUMMARY  AND  KEY 

syphilis.  This  patient  may  well  have  been  a  moron  at  the  out- 
set and  exhibited  some  reactions  (refusal  to  talk)  explicable  on 
the  basis  of  feeblemindedness.  She  was  a  neurosyphilitic  only 
in  the  sense  of  the  neural  complication  that  we  find  in  the 
secondary  stage  of  syphilis.  As  stated  above,  we  do  not  yet 
know  what  the  fate  of  these  neural  complications  of  secondary 
syphilis  is  to  be.  The  frequency  of  this  finding  in  secondary 
syphilis  is  probably  too  great  to  warrant  the  hypothesis  that 
it  must  always  go  on  to  a  chronic  neurosyphilis ;  but  we 
certainly  are  warranted  in  regarding  these  cases  as  potential 
chronic  neurosyphilitics. 

A  case  of  TABOPARETIC  NEUROSYPHILIS  in  which  the  heavy 
exudate  characteristic  of  paresis  became  a  soil  for  a  growth 
of  the  typhoid  bacillus  is  presented  with  autopsy.*  This 
fatality  with  TYPHOID  MENINGITIS  is  merely  a  concrete  ex- 
ample of  the  many  complications  which,  syphilitics  and  especi- 
ally neurosyphilitics  have  to  sustain. 

The  case  series  then  goes  on  to  illustrate,  though  quite 
inadequately,  a  variety  of  MEDICOLEGAL  AND  SOCIAL 
complications  of  neurosyphilis.  It  is  well  known  that  many 
social  complications  with  grave  moral,  economic  and  even 
political  difficulties  occur. 

Our  series  starts  with  a  "  public  character  "f  whose  elo- 
quence and  reformatory  efforts  led  to  a  considerable  noto- 
riety. The  autopsy  in  this  case  showed  singularly  few  lesions 
despite  the  fact  that  the  case  was  microscopically  one  of 
wholly  characteristic  PARETIC  NEUROSYPHILIS.  The  ques- 
tion might  arise  how  far  we  are  entitled  to  correlate  the  refor- 
matory efforts  of  this  always  eccentric  character  with  syphilis. 
The  man  himself  a  physician,  was  aware  of  the  doubt  which  his 
Argyll-Robertson  pupils  threw  upon  his  medical  situation. 
He  explained  them  on  the  basis  of  an  old  smallpox!  We  are 
inclined  to  think  that  the  whole  of  this  man's  life,  from  his 
giving  up  of  medical  practice  to  live  as  a  kind  of  literary  and 
political  hack,  was  due  to  subtle  changes  of  neurosyphilitic 

*  Frederick  Estabrook  (82). 

t  Maj.  Isaac  Thompson,  M.D.  (83). 


SUMMARY  AND   KEY  455 

origin.  The  fact  that  there  was  a  certain  delinquent  streak 
in  the  man  is  not  inconsistent  with  this  idea.  Interestingly 
enough,  a  fall  on  the  ice  in  the  man's  6ist  year  actually 
started  up  the  fatal  process,  a  condition  of  affairs  amply 
illustrated  in  cases  of  neurosyphilis,  brought  out  by  trauma 
that  come  to  the  attention  of  the  Industrial  Accident  Board 
in  connection  with  claims  for  compensation. 

A  case  of  sudden  grandiosity*  illustrates  an  episode  of 
NEUROSYPHILITIC  origin.  Such  a  person  might  well  be  re- 
garded by  the  lay  newspaper  reader  as  a  crank  or  a  grafter  but 
the  neurosyphilitic  possibility  should  always  be  entertained 
in  cases  of  this  order. 

As  against  the  social  difficulties  that  look  in  the  direction 
of  the  classical  paretic  grandeur,  we  present  a  case  of  apparent 
suicidal  attempt  by  gas,  which  attempt  was  followed  by  a 
period  of  amnesia  that,  taking  into  account  the  laboratory 
findings,  was  probably  NEUROSYPHiLiTic.f 

Vistas  of  extraordinary  interest  are  opened  out  by  studies 
of  the  relation  of  neurosyphilis  to  delinquency.  The  case 
of  the  psychopathic  reformer  (Case  83)  above  mentioned  was 
one  in  which  the  delinquency  may  possibly  have  been  related  to 
acquired  syphilis.  We  present  also  a  case  of  juvenile  neuro- 
syphilis, a  young  man  of  reform  school  type  |  in  which  JUVE- 
NILE PARETIC  NEUROSYPHILIS  was  established.  This  patient, 
in  fact,  deteriorated  very  rapidly  to  a  condition  of  considerable 
dementia  a  few  months  after  the  diagnosis  was  established. 

A  striking  case  of  so-called  DEFECTIVE  DELINQUENCY  is 
presented,  an  alcoholic  prostitute  of  the  reformatory  group.  § 
The  NEUROSYPHILIS  in  this  case  was  a  complication  rather 
than  an  original  factor  in  the  delinquency. 

One  case  of  PARESIS  SINE  PARESI  was  that  of  an  habitual 
criminal  |j  and  forger  who,  without  showing  mental  or  physical 
symptoms  of  neurosyphilis,  yielded  the  laboratory  signs  of 
paretic  neurosyphilis.  Again,  as  in  the  case  of  the  prostitute 

*  Lester  Smith  (84).  §  Vivian  Walker  (87). 

f  Annie  Marks  (85).  ||  -  -  (88).    Cf. 

Richard  Lawlor  (25). 
J  Frank  Johnson  (86).  Bessie  Vogel 


456  SUMMARY  AND   KEY 

just  mentioned,  the  CRIMINALITY  seems  to  have  antedated 
the  neurosyphilis  and  even  to  have  been  hereditary. 

By  way  of  introducing  the  next  group  of  Industrial  Ac- 
cident Board  cases,  we  present  a  case  of  JUVENILE  PARESIS 
with  initial  TRAUM  . 

The  Industrial  Board  group  is  of  note  in  that  the  signs  of 
the  traumatic  formf  of  paretic  neurosyphilis  do  not  occur 
immediately  upon  the  accident.  Some  time  elapses  in  which 
the  physical,  chemical  or  parasitological  changes  have  time 
to  work  themselves  out  in  the  injured  tissues.  Many  hy- 
potheses may  be  raised  as  to  the  reason  why  a  trauma  lights 
up  a  syphilitic  process.  Of  course,  false  claims  J  may  be 
made  for  compensation  by  neurosyphilitics  in  whom  the 
symptoms  were  already  in  existence  before  the  accident  and 
in  whom  they  may  not  even  be  markedly  exacerbated  by  the 
accident.  The  false  claimants  can  probably  not  readily 
frame  a  story  which  the  expert  psychiatrist  cannot  discredit 
if  he  is  allowed  to  perform  laboratory  tests  and  give  the  patient 
the  benefit  of  thorough  examination.  However,  some  cases 
of  established  PARETIC  NEUROSYPHILIS  are  perhaps  truly 
subject  to  exacerbations  §  of  the  clinical  process  and  it  may 
well  be  held  that  such  exacerbations  warrant  partial  com- 
pensation. 

The  fact  that  a  trauma  may  light  up  a  syphilitic  process 
is  illustrated  in  a  case  that  came  to  the  Psychopathic  Hospital, 
in  which  a  SYPHILITIC  LESION  developed  in  the  skull  AT  THE 
SITE  OF  SKULL  INJURY.  || 

A  case  of  OCCUPATION-NEUROSIS  ^[  that  might  be  interpreted 
as  a  syphilitic  neuritis  is  presented.  The  case  is  still  in  doubt 
as  to  its  scientific  evaluation. 

The  workmen's  compensation  group  of  syphilitic  cases  is 
of  extraordinary  general  interest  since  it  indicates  that 

*  Margaret  Tennyson  (89).       f  Joseph  O'Hearn  (90). 
John  Lawrence  (5).  +  T      •  c 

LeV1  Sussman 


Mary  Coughlin  (35). 

Theresa  Mullen  (36).  §  Joseph  Larkin  (92). 

John  Friedreich  (77).  n  T>-  1      JTV/T      u  «</     \ 

Gridley  Ringer  (78).  1  1  Richard  Marshall  (93). 

James  Arnold  (80).  If  David  Fitzpatrick  (94). 


SUMMARY  AND  KEY  457 

employers  may  well  be  on  the  lookout  not  to  employ  known 
syphilitics  unless  fortified  by  special  insurance  arrangements. 
Whether  in  future  employers  may  desire  to  employ  only  W.  R. 
negative  workmen  is  one  of  the  highly  complicated  questions 
re  workmen's  compensation  and  health  insurance. 

But  the  problems  of  neurosyphilis  are  not  merely  medico- 
legal  and  broadly  public  or  social.  The  most  appealing  dif- 
ficulties lodge  within  the  bosom  of  the  family.  Now  and 
then  a  case  of  INCOMPATIBILITY  OF  TEMPERAMENT,  perhaps 
complicated  by  alcoholism,  occurs  which  tests  prove  to  be 

NEUROSYPHILITIC.* 

Special  attention  should  be  drawn  to  a  certain  NEURO- 
SYPHILITIC FAMILY  f  in  which  both  parents  and  five  children 
showed  a  variety  of  syphilitic  diseases,  including  syphilis 
without  apparent  neural  complications,  paretic  neurosyphilis, 
juvenile  paresis,  aortic  aneurysm,  achondroplasia  and  caries 
of  the  spine,  and  an  as  yet  indefinite  neurosis.  There  was  a 
sixth  child  that  died  shortly  after  birth,  as  well  as  three  still- 
born. 

One  cannot  conclude  from  the  normal  t  look  of  a  neuro- 
syphilitic's  family  that  the  normal  looking  members  are  not 
syphilitic,  as  illustrated  by  the  family  of  our  draughtsman. 

The  most  intricate  social  complications  may  arise.  We 
present  a  case  of  a  syphilitic  man  (a  well-to-do  merchant) 
who  was  apparently  being  goaded  into  a  second  marriage  § 
because  he  was  continually  being  charged  with  having  caused 
his  first  wife's  death.  This  he  had  actually  done  in  a  certain 
sense  because  his  wife  had  died  of  general  paresis,  having 
contracted  syphilis  from  him. 

In  the  fifth  section  on  THERAPY,  we  have  attempted  to 
outline  some  of  the  principles  and  problems  that  arise  in  the 
treatment  of  neurosyphilis.  Enough  has  probably  been  said 

*  Joseph  Wilson  (95).  %  Walter  Heinmas  (97). 

f  Becky  Bornstein  (96).        §  Mr.  Jacobs  (98). 
Walter  Heinmas  (97). 
Mr.  Jacobs  (98). 


458  SUMMARY   AND   KEY 

concerning  the  attitude  of  optimism  or  pessimistic  nihilism 
that  may  be  adopted  toward  the  whole  subject.  It  must  be 
borne  in  mind,  however,  that  a  great  deal  of  the  work  on 
treatment  of  neurosyphilis  is  still  in  the  experimental  stage. 
As  a  rule,  each  case  must  be  considered  separately  and  in- 
dividually and  the  prognosis  can  be  made  satisfactorily  only 
after  treatment  has  been  given.  This  section  contains  a 
group  of  cases  that  have  been  treated  rather  intensively  and 
the  results  of  this  treatment  are  indicated.  The  section  is 
introduced  by  five  untreated  cases,  the  brains  and  cords  of 
which  have  been  studied  post  mortem.  These  illustrate  the 
pathological  conditions  which  we  have  to  meet,  and  from 
these  examples  we  can  draw  the  theoretical  conclusion  that 
some  cases  are  beyond  the  aid  of  therapy  on  account  of  the 
brain  destruction.  Others,  in  which  the  symptomatology 
bespeaks  just  as  grave  a  situation,  turn  out  on  autopsy  to  have 
very  little  actual  damage  to  the  brain  tissues  and  therefore 
should  theoretically  at  any  rate  be  amenable  to  antisyphilitic 
therapy. 

In  order  to  get  any  adequate  conception  of  the  possibilities 
of  therapeutic  results  in  cases  of  neurosyphilis,  one  must 
consider  the  pathological  changes  that  occur  and  how  far 
these  changes  are  reparable.  In  cases  in  which  the  destruc- 
tion of  tissue  is  marked,  it  is,  of  course,  out  of  the  question  to 
expect  to  get  any  marked  clinical  improvement.  A  case  of 
spastic  hemiplegia  *  in  paretic  neurosyphilis  is  given  with  the 
autopsy  findings  as  an  illustration  of  irreparable  damage 
that  may  occur  to  the  parenchymatous  structure,  thus  pre- 
cluding any  chance  of  functional  recovery. 

On  the  other  hand,  there  is  a  group  of  cases  in  which  the 
symptoms  may  be  exceedingly  severe  and  yet  the  actual  de- 
struction of  tissue  be  almost  nil.  This  point  is  illustrated  by 
a  casef  in  which  total  duration  of  symptoms  terminating  in 
death  was  only  22  days.  At  autopsy  there  was  very  little  in 
the  way  of  macroscopical  lesions,  and  microscopically  there 
was  no  marked  evidence  of  destruction  in  the  parenchymatous 

*  James  McDevitt  (99). 
f  Jacob  Methuen  (100). 


SUMMARY  AND   KEY  459 

tissue.  The  lesions  were  represented  chiefly  by  perivascular 
infiltration.  According  to  all  our  modern  ideas,  this  type  of 
reaction  is  resolvable  under  antisyphilitic  treatment.  Though 
this  case  was  one  of  very  short  duration,  similar  pathological 
pictures  may  be  obtained  in  cases  of  considerably  longer 
standing.  It  is  also  of  great  importance  to  remember  that 
symptomatically  such  a  case  may  be  in  no  way  distinguished 
from  a  case  with  marked  atrophy. 

Another  autopsied  case  is  given  which  shows  an  exceedingly 
marked  meningitis.*  The  meningitic  processes  according  to 
the  literature  and  experience  react  very  readily  to  antisyphi- 
litic treatment  in  the  form  either  of  mercury  and  iodid  or  in 
combination  with  salvarsan.  The  lesion  here  present  would 
probably  have  improved  had  intensive  treatment  been  given. 
Clinically  the  diagnosis  of  general  paresis  was  made  and,  as 
has  been  the  rule  in  the  past,  treatment  was  not  given  on 
the  ground  that  it  had  no  value  in  paresis.  While  this  is  an 
extreme  case  of  meningitis,  it  is  to  be  remembered  that  the 
vast  majority  of  cases  of  paretic  neurosyphilis  show  some  de- 
gree of  meningitis.  Just  as  in  the  marked  meningitis  of  the 
diffuse  neurosyphilis,  so  with  the  meningitis  of  the  paretic 
form,  improvement  is  expected  under  treatment.  As  a  part 
or  even  the  whole  of  the  symptomatology  in  a  given  case  may 
be  due  to  this  meningitic  process,  we  have  reason  occasionally 
to  expect  marked  improvement  as  the  result  of  antisyphilitic 
treatment. 

As  a  contrast  to  this  case  with  marked  meningitis,  another 
case  of  marked  atrophy  f  is  given.  Here  the  atrophy  was  very 
perceptible  on  macroscopical  examination  and  the  mere  view 
of  the  brain  at  once  indicated  that  in  such  a  case  important 
results  from  treatment  were  not  to  be  expected. 

The  topographical  variation  of  the  lesions  in  neurosyphi- 
lis must  be  remembered  when  treatment  is  to  be  instituted. 
Thus  very  marked  lesions  may  exist  in  portions  of  the  brain 
which  do  not  give  any  very  definite  localizing  symptoms.  As 
a  result,  one  may  be  led  to  believe  from  clinical  evidence 
that  the  case  is  a  very  mild  one  though  the  lesions  may 

*  John  Baxter  (101).        f  Theodosia  Jewett  (102). 


460  SUMMARY  AND  KEY 

really  be  very  extensive.  The  topographical  distribution 
must,  therefore,  be  taken  into  consideration  in  trying  to 
estimate  the  damage  done.  This  point  of  topographical  dis- 
tribution of  the  lesions  is  illustrated  by  a  case.* 

It  has  been  generally  recognized  that  clinical  improvement, 
if  not  cure,  may  be  readily  obtained  in  the  group  of  diffuse 
neurosyphilis,  i.e.,  so-called  cerebral  and  cerebrospinal  forms 
of  syphilis.  These  are  cases  in  which  the  parenchyma  is  very 
slightly,  if  at  all,  affected  and  in  which  the  lesion  is  chiefly 
in  the  meninges  and  blood  vessels,  irritative  rather  than 
degenerative.  A  casef  is  given  to  illustrate  this  point.  In 
our  experience  systematic  intravenous  salvarsan  therapy 
associated  with  mercury  and  iodid  gives  remarkably  good 
results  in  the  vast  majority  of  this  group  of  cases. 

It  is  generally  conceded  that  antisyphilitic  treatment, 
particularly  salvarsan,  has  a  very  satisfactory  result  applied 
to  diffuse  neurosyphilis.  But  the  same  good  results  may  be 
obtained  in  cases  which  are  not  so  typically  of  the  diffuse 
type.  An  illustration  is  given  in  the  case  of  a  machinist  in 
which  the  diagnosis  was  in  doubt  between  paretic,  tabetic  or 
diffuse  neurosyphilis. $  The  result  of  treatment  was  as  satis- 
factory as  could  be  expected  in  any  type  of  neurosyphilis 
and  this  in  a  case  of  several  years'  duration  with  Argyll- 
Robertson  pupils. 

As  a  rule,  the  Argyll-Robertson  pupil  is  taken  as  a  grave 
omen  for  treatment,  an  idea  based  upon  a  conception  that 
the  Argyll- Robertson  pupil  so  frequently  represents  the  old 
so-called  "  parasyphilitic  "  cases,  which,  in  the  past  were 
taught  as  being  incapable  of  improvement  by  the  ordinary 
antisyphilitic  methods. 

A  second  case  §  with  Argyll-Robertson  pupil  shows  again 
that  the  prognosis  may  be  very  good  despite  the  Argyll- 
Robertson  sign. 

*  A.  W.  (103).  Agnes  O'Neil  (19), 

t  John  Edwards  (104).     Cf.        Paul°  Marini  <28)' 

Henri  Lepere  (105),  |  Henri  Lepere  (105).     Cf. 
Frederick  Stone  (106),  Julius  Kantor  (54). 

Arthur  Bright  (121),  §  Frederick  Stone  (106). 


SUMMARY  AND  KEY  461 

But  even  in  the  diffuse  neurosyphilis,  the  symptomatic  re- 
sults of  treatment  may  not  be  entirely  happy.  Under  treat- 
ment it  may  be  possible  to  reduce  the  spinal  fluid  tests  to 
negative  without,  however,  as  in  the  case  of  our  hemiplegic 
lady,*  making  the  physical  or  mental  symptoms  disappear. 
In  other  words,  it  may  be  possible  to  stop  the  active  prog- 
ress of  the  disease  without  removing  the  symptoms. 

One  is  always  warned  of  the  danger  of  intravenous  salvarsan 
therapy  in  hemiplegic  cases  due  to  arteriosclerotic  conditions. 
While  this  warning  is  well  justified,  it  does  not  mean  that  the 
most  intensive  treatment  is  contraindicated,  as  shown  in  the 
case  of  our  hemiplegic  machinist.f  Such  may  be  given  over 
long  periods  of  time  with  the  most  satisfactory  results. 

A  case  J  is  given  which  illustrates  the  value  of  antisyphilitic 
treatment  in  cases  showing  symptoms  of  intracranial  pres- 
sure due  to  syphilitic  disease.  In  the  case  of  the  woman  which 
we  cite,  we  believe  that  the  symptoms  of  intracranial  pressure 
were  probably  due  to  a  gummatous  new  growth,  although  it  is 
possible  that  they  were  due  to  a  marked  meningitic  process. 
However,  the  results  of  a  limited  amount  of  antisyphilitic 
treatment  in  this  case  were  very  brilliant.  Similar  results 
may  often  be  obtained  in  gumma  of  the  brain.  This  is  not 
always  true,  however,  and  it  may  become  necessary  to  use 
surgical  procedure  in  order  rapidly  to  overcome  the  effects  of 
intracranial  pressure. 

While  it  has  always  been  conceded  that  treatment  would 
greatly  help  cases  of  diffuse  and  vascular  neurosyphilis,  the 
utmost  pessimism  has  existed  concerning  the  results  to  be 
obtained  by  treatment  in  cases  of  tabetic  and  paretic  neuro- 
syphilis. Only  in  the  last  five  or  six  years,  due  to  the  stimulus 
of  Ehrlich's  discovery  of  salvarsan  and  the  introduction  of  the 
intraspinous  methods  of  therapy,  have  intensive  work  and 
study  been  given  to  the  treatment  of  these  cases.  And  though 
it  has  been  by  no  means  settled  in  the  minds  of  the  various 
workers  in  this  field,  as  to  what  the  ultimate  results  of  such 

*,Greta  Meyer  (107).     Cf.     t  Victor  Friedburg  (108). 
John  Jackson  (14).  j  Annie  Rivers  (109). 


462  SUMMARY   AND    KEY 

treatment  will  be  and  though  some  do  not  believe  that  there  is 
any  good  to  be  expected  from  our  present  methods,  still  the 
majority  of  men  who  are  treating  these  cases  systematically 
feel  very  much  encouraged. 

At  times  very  brilliant  results  are  to  be  obtained  by  in- 
traspinous  treatment  in  tabetic  neurosyphilis  ("  tabes  dor- 
salis  ").  A  very  striking  illustration  is  given  of  a  case  of 
this  sort  in  which  the  symptoms  dated  only  a  few  months  but 
which  had  all  the  classical  symptoms,  signs  and  laboratory 
tests.  Five  intraspinous  injections  of  mercurialized  serum 
were  sufficient  to  cause  the  disappearance  of  the  subjective 
symptoms  and  to  reduce  the  spinal  fluid  test  to  negative.* 

It  must  be  emphasized  that  the  best  results  in  cases  of 
tabetic  neurosyphilis  are  usually  to  be  expected  in  cases  in 
which  the  symptoms  are  of  short  standing.  Where  the  proc- 
ess is  of  long  duration  and  much  destruction  of  spinal  cord 
tissue  has  occurred,  the  best  one  can  expect  is  that  the  ac- 
tivity and  progress  may  be  halted.  This  is  illustrated  by  our 
case  of  a  baker,  43  years  of  age,  who  had  been  suffering  from 
the  symptoms  of  tabes  for  some  years.  Under  treatment  it 
was  possible  to  get  an  entirely  negative  serology  of  the  blood 
and  spinal  fluid,  f  Despite  this  evidence  that  the  activity 
of  syphilis  had  ceased,  the  symptoms  continued  unabated. 
We  are  ready  to  believe,  however,  that  much  good  was  ac- 
complished. For  the  patient  should  not  have  any  further 
untoward  developments  or  the  appearance  of  any  new 
symptoms.  These,  without  such  treatment,  might  well  be 
expected.  At  times  excellent  clinical  results  are  obtained  in 
long  standing  cases. 

The  results  of  treatment  in  paretic  neurosyphilis  ("  general 
paresis  ")  have  been  considered  even  less  hopeful  than  in 
tabetic  neurosyphilis  ("tabes  dorsalis ") ;  indeed,  it  has 
often  been  stated  that  the  patients  are  made  worse  by 
treatment.  Recent  work,  however,  supports  a  much  more 
optimistic  viewpoint.  We  feel  that  intensive  treatment  has 
been  of  the  greatest  value  in  a  number  of  cases  of  paretic 

*  Mr.  McKenzie  (no).    Cf.      t  Ivan  Rokicki  (in). 
Ivan  Rokicki  (in). 


SUMMARY  AND   KEY  463 

neurosyphilis.  Two  cases  are  given  which  show  the  most  satis- 
factory and  brilliant  results  of  intensive  intravenous  salvarsan 
therapy  in  cases  diagnosed  as  general  paresis.  The  first  case, 
an  excellent  salesman,  46  years  of  age,  with  most  aggravated 
mental  symptoms,  recovered  symptomatically  and  all  his 
tests  were  rendered  negative.*  He  has  now  remained  entirely 
well  and  economically  efficient  for  about  two  years  without 
further  treatment.  The  other  case,  f  a  housewife,  also  with 
very  marked  symptoms  suggestive  in  all  ways  of  general 
paresis,  also  recovered  rapidly  under  treatment  and  her 
tests  became  negative.  Her  remission  has  now  lasted  for 
nearly  three  years  without  further  treatment. 

At  times  it  is  not  possible  to  get  the  spinal  fluid  tests  to 
become  negative  in  cases  of  paretic  neurosyphilis  under  the 
most  intensive  salvarsan  therapy.  In  spite  of  this,  the 
clinical  condition  of  the  patient  may  improve  so  greatly  that 
the  patient  can  be  considered  clinically  recovered.  An  illus- 
tration is  given  of  an  undertaker  J  who  was  brought  from  a 
condition  of  the  greatest  cachexia  and  mental  confusion  to  a 
condition  of  robust  appearance  and  mental  efficiency  under 
intravenous  salvarsan  therapy,  in  spite  of  the  fact  that  his 
tests  were  very  slightly  if  at  all  reduced  in  intensity.  He 
has  been  able  to  resume  his  former  occupation  and  his  former 
life  with  great  satisfaction  to  himself  and  his  family. 

Improvement  in  paretic  neurosyphilis  under  treatment  is 
not  to  be  expected  very  early.  Two  or  three  months  of 
active  treatment  may  elapse  before  one  sees  signs  of  improve- 
ment. Indeed,  as  illustrated  by  our  case  of  the  shipping  clerk, 
this  improvement  may  begin  to  make  its  appearance  only 
after  more  than  four  months  of  intensive  treatment  consisting 
of  two  injections  of  salvarsan  per  week.§  In  spite  of  the  long 
delay  in  this  case,  complete  clinical  recovery  occurred  and  the 
tests  became  almost  negative  at  the  end  of  a  year  of  treatment. 

*  Albert  Forest  (112).   Cf.        Levi  Morovitz  (122), 
Gussie  Silverman  (113),         Peter  Burkhardt  (58). 

Walter  Henry  (114)  f  Gussie  Silverman  (113). 

William  Rosetti  (116), 

Annie  Martin  (117),  t  Walter  Henry  (JI4)- 

§  Henry  Ryan  (115). 


464  SUMMARY  AND   KEY 

It  is  not  only  in  the  central  nervous  system  that  the 
syphilitic  process  may  resist  the  most  intensive  treatment. 
In  the  case  of  the  speculator,  a  victim  of  paretic  neurosyphilis, 
which  we  cite,  a  perennially  recurrent  iritis  appeared  after 
several  months  of  the  most  intensive  salvarsan  treatment 
which  was  apparently  sufficient  to  reduce  the  symptoms  of 
the  paretic  neurosyphilis,*  but  not  of  non-neural  syphilis. 

We  give  the  case  of  a  charwoman  having  the  diagnosis  of 
paretic  neurosyphilis,  who,  under  intensive  treatment,  made  a 
symptomatic  recovery.  The  interesting  point  in  her  findings 
is  that  all  the  tests  in  the  spinal  fluid  became  negative  except 
the  gold  sol  reaction  which  remained  of  the  "  paretic  "  type.f 
There  is  no  general  rule  as  to  the  reaction  of  the  spinal  fluid 
tests  under  treatment.  At  times  one  test  is  the  first  to  disap- 
pear under  treatment;  again  it  is  another.  We  have  seen 
many  cases  in  which  the  gold  sol  was  the  first  test  to  become 
negative  and  others,  as  the  case  given,  in  which  it  is  the  last  to 
show  any  change.  As  in  our  undertaker,  symptomatic  clini- 
cal improvement  may  be  practically  complete  without  any 
change  in  the  spinal  fluid  tests. 

One  must  remember  that  it  is  the  condition  of  the  patient 
that  is  of  first  importance;  not  so  much  the  laboratory  tests. 
Having  shown  the  clinical  recoveries  with  the  tests  remaining 
positive,  we  now  have  to  report  two  cases  in  which  there 
was  improvement  as  shown  by  the  tests  but  no  clinical  im- 
provement. The  first  patient,  a  bank  tellerf  of  39  years,  with 
a  diagnosis  of  paretic  neurosyphilis,  received  intensive  intra- 
venous salvarsan  for  several  months.  Under  this  treatment 
all  the  tests  became  negative  except  the  gold  sol  which  re- 
mained of  the  paretic  type.  In  spite  of  this,  there  was  not 
the  slightest  improvement  in  his  mental  condition. 

The  second  case,  a  young  man  of  29  years  in  whom  the  symp- 
toms of  neurosyphilis  had  recently  appeared,  under  treatment 
showed  a  marked  diminution  in  the  intensity  of  the  spinal 


*  William  Rosetti  (116).         J  William  Roberts  (118). 

John  Silver  (119). 
t  Annie  Martin  (117).    Cf. 

William  Roberts  (118). 


SUMMARY  AND  KEY  465 

fluid  tests,  notwithstanding  which  the  patient  became  more 
and  more  demented  and  died  after  a  series  of  convulsions.* 

Of  course,  good  results  indicated  above  in  some  of  our  cases 
of  paretic  neurosyphilis  are  not  to  be  expected  in  every  case 
no  matter  how  intensive  the  treatment.  We  give  a  case  of 
paretic  neurosyphilis  in  which  the  most  intensive  intravenous 
salvarsan  therapy  gave  no  satisfactory  results.  This  was 
followed  by  several  intraventricular  injections  of  salvarsan- 
ized  serum.  The  results  of  this  combined  treatment,  however, 
were  still  not  satisfactory,  and  the  patient  died.f 
{fin  order  to  emphasize  as  strongly  as  possible  what  we  be- 
lieve is  a  great  advantage  of  systematic  intensive  treatment 
for  neurosyphilis,  we  offer  two  cases  in  different  time  periods 
of  neurosyphilis.  The  first  is  a  printer  with  the  symptoms  of 
diffuse  neurosyphilis  six  months  after  the  appearance  of  his 
chancre.  J  These  symptoms  appeared  despite  three  injections 
of  salvarsan,  injections  of  mercury  and  mercury  by  mouth. 
Under  intensive  treatment  (meaning  injections  of  salvarsan 
twice  a  week  and  continued  injections  of  mercury),  complete 
recovery  occurred  in  a  few  weeks. 

The  second  case  is  that  of  a  waiter  with  signs  and  symptoms 
of  neurosyphilis  in  whom  the  diagnosis  lay  between  the  diffuse 
and  paretic  forms.  §  This  patient  developed  his  symptoms 
in  spite  of  continuous  antisyphilitic  treatment  during  the  six 
years  since  his  infection.  This  treatment  had  been  compar- 
atively mild,  consisting  in  great  part  of  mercury  by  mouth. 
However,  he  had  had  courses  of  injections  of  mercury  and 
several  injections  of  salvarsan.  Under  a  systematic  course  of 
intravenous  injections  of  salvarsan  twice  a  week  for  a  number 
of  months,  all  symptoms  disappeared  and  the  spinal  fluid  tests 
became  negative  as  well  as  the  W.  R.  in  the  blood  serum. 

A  final  case  is  offered  which  indicates  that  antisyphilitic 
treatment  may  occasionally  be  of  service  in  improving  the 
mentality  of  a  FEEBLEMINDED  CONGENITAL  SYPHILITIC.)  | 

*  John  Silver  (119).  John  Bennett  (34). 

f  James  McGinnis  (120).  §  Levi  Morovitz  (122). 

J  Arthur  Bright  (121).     Cf.      ||  Robert  Matthews  (23).  Cf. 
Levi  Morovitz  (122),  Isaac  Goldstein  (37). 


466  SUMMARY   AND    KEY 

No  attempt  has  been  made  in  this  section  to  give  a  per 
cent  evaluation  of  the  results  of  treatment  in  any  one  group  of 
neurosyphilis.  Two  charts  (charts  25  and  26),  however,  are 
appended  which  give  an  indication  of  some  of  our  results.  It 
seems  to  us,  however,  that  it  is  too  early  to  make  any  definite 
statements  as  to  how  far  treatment  will  take  us  in  the  groups 
of  neurosyphilis.  We  do  feel  decidedly,  however,  that  many 
patients,  in  whatever  group  of  neurosyphilis  the  diagnosis 
may  place  them,  will  respond  to  intensive  systematic  anti- 
syphilitic  treatment.  It  is  unfair  to  give  an  entirely  grave 
prognosis  in  any  case  of  neurosyphilis  until  the  effect  of 
treatment  has  been  tried. 

In  a  separate  section,  entitled  NEUROSYPHILIS  AND 
THE  WAR,  we  have  presented  fourteen  cases  selected  from 
British,  French  and  German  writers  in  the  war  literature  of 
1914-16.  Most  of  these  cases  were  naturally  somewhat  inad- 
equately reported  under  the  critical  conditions  of  literature 
made  in  the  war.  We  present  the  cases  for  what  they  are 
worth :  at  all  events  they  draw  attention  to  the  extraordinary 
interest  of  the  neurosyphilis  problem  in  relation  to  the  war. 

Such  cases  as  A,  one  of  tabes  dorsalis  apparently  develop- 
ing paresis  by  a  process  akin  to  shell-shock,  is  of  value  in  the 
interpretation  of  the  development  of  paresis  in  civil  life. 
By  "  shell-shock  "  we  commonly  refer  to  a  condition  in  which 
there  is  no  actual  traumatic  injury  of  the  brain.  The  hy- 
pothesis must  be  then  that  the  explosion  in  some  way  indi- 
rectly caused  an  alteration  of  living  conditions  of  the  spiro- 
chetes,  permitting  the  development  of  paresis. 

Case  B  similarly  seems  to  be  a  case  in  which  a  latent  syphi- 
lis has  turned  shell-shock  into  tabes  dorsalis. 

Cases  C,  D,  E  bring  up  the  question  of  aggravation  of 
neurosyphilis  by  service  and  on  service,  respectively. 

Case  F  likewise  shows  how,  in  the  determination  of  amount 
of  pension,  the  probable  duration  of  the  neurosyphilitic  pro- 
cess is  important. 

Case  G  seems  to  shoW  that  war  stress^  alone,  without  the 
emotional  or  physical  effects  of  shell-shock,  may  kindle  a 
latent  syphilis  into  paretic  neurosyphilis. 


SUMMARY  AND  KEY  467 

Case  H  similarly  suggests  that  'the  ''gassing"  process  may 
effect  the  same  result. 

Case  I  seems  to  show  that  the  neuropathically  tainted 
person  may  have  latent  epilepsy  brought  out  through  syphilis, 
the  syphilis  in  this  case  having  been  acquired  during  the  first 
summer  of  the  war. 

Case  J  was  an  interesting  case  of  a  syphilitic  who,  after 
the  stress  of  the  Battle  of  Dixmude,  became  an  epileptic. 

Syphilitic  root-sciatica  was  developed  in  Case  K  at  work 
in  the  war  zone. 

Case  L  is  one  of  a  civilian  who  apparently  would  not  have 
developed  paresis  at  precisely  the  moment  when  he  did,  if 
he  had  not  been  discharged  as  a  German  Jew  from  his  long- 
held  bank  position  in  London. 

Two  cases,  M  and  N,  are  cases  of  shell-shock,  non-syphi- 
litic; yet  the  picture  of  paresis  in  the  one  case  and  of  tabes 
in  the  other  was  for  a  long  time  almost  convincing  to  the 
examiners.  They  are  better  termed  cases  of  pseudoparesis 
and  pseudotabes,  using  the  prefix  "pseudo",  as  usual,  to 
signify  a  non-syphilitic  imitation  of  the  disease  in  question. 

To  sum  up  in  the  most  general  way  the  lessons  of  this 
book,  we  may  emphasize  again  (i)  the  unity -in-variety  of  the 
phenomena  of  neurosyphilis,  (2)  the  value  of  a  hopeful  approach 
to  the  therapy  of  all  cases  of  neurosyphilis,  even  the  parelic  form, 
and  (3)  the  value  of  applying  syphilis  tests  to  every  case  of  neuro- 
sis or  psychosis. 

(i)     RE  unity -in-variety  of  neurosyphilitic  phenomena. 

The  unity  of  these  phenomena  is  confirmed,  theoretically, 
by  the  common  factor  of  spirochetosis :  practically,  by  the 
Wassermann  reaction,  positive  in  serum  or  spinal  fluid!  Al- 
most at  this  point  the  unity  of  phenomena  ceases.  Neither 
chronicity,  nor  evidence  of  mononuclear  cell-deposits,  nor 
evidence  of  serious  structural  damage  to  the  nervous  system, 
nor  presence  of  other  positive  tests  than  the  W.  R.,*  nor 

*  For  cases  in  which,  without  autopsy  we  have  risked  the 
diagnosis  neurosyphilis  in  the  absence  of  W.  R.  in  serum  or 
fluid,  see  William  Twist  (13),  Frederick  Wescott  (18),  Martha 
Bartlett  (21),  Thomas  Donovan  (23),  Paolo  Marini  (28), 
Margaret  Neal  (32),  Bridget  Curley  (59),  Victor  Friedburg 
(108),  Ivan  Rokicki  (HI). 


468  SUMMARY  AND  KEY 

existence  of  mental  or  nervous  symptoms  or  signs,  is  a  com- 
mon feature  of  neurosyphilis.  Sometimes  the  nervous  system 
appears  to  harbor  spirochetes  in  the  most  cordial  manner  as 
guest-friends  (paresis  sine  paresi.)  Again,  perhaps  as  an 
expression  of  elaborate  processes  of  immunity,  the  spi- 
rochetes take  effect  in  relatively  huge  gummata.  Sometimes 
the  neurosyphilitic  process  rises  as  if  by  a  regular  process  of 
siege  from  spinal  nerve-root  to  spinal  nerve-root  (tabes 
dorsalis  and  diffuse  neurosyphilis).  Again,  the  nervous 
system  is  taken  by  storm,  as  it  were  (disseminated  encepha- 
litis). Very  frequently  the  neurosyphilis  is  simply  an  in- 
direct effect  of  blood-vessel  disease,  and  huge  masses  of 
tissue  are  scooped  out  in  necrosis  with  dependent  secondary 
degenerations ;  and  later  the  extinct  lesions  of  vascular  origin 
may  or  may  not  betray  evidence  of  their  syphilitic  origin. 
Sometimes  diffuse  processes  run  on,  apparently,  with  perfect 
fatalism  to  a  mortal  issue  in  a  few  years  both  with  and  without 
treatment.  Again  treatment  appears  to  accomplish  much 
(see  fuller  discussion  under  2).  The  laws  governing  the  pref- 
erence of  processes  to  lodge  in  membranes,  vessels,  and 
parenchyma,  and  in  all  combinations  of  these,  have  not  been 
worked  out.  Hardly  a  case  of  neurosyphilis,  properly  studied 
ante  mortem  and  post  mortem,  but  would  throw  important 
light  on  our  medical  approach  to  one  of  the  great  problems 
of  civilization,  the  problem  of  syphilis  as  a  whole. 

(2)  RE  value  of  a  hopeful  approach  to  the  therapy  of  neuro- 
syphilis. 

The  prognosis  of  neurosyphilis  is  not  worse  than  that  of 
the  chronic  diseases  in  general.  In  fact,  the  prognosis  of 
neurosyphilis  quoad  vitam  is  either  good  or  dubious,  certainly 
not  bad.  The  surprising  reversals  of  form  which  the  spi- 
rochete  shows  in  certain  remissions  are  always  to  be  awaited. 
Treatment  of  neurosyphilis  has  certainly  effected  amazing 
results,  not  so  much  by  way  of  Ehrlich's  therapia  sterilisans 
magna  as  by  means  of  systematic  intensive  treatment. 
Even  paretic  neurosyphilis  (general  paresis)  seems  to  have 
been  cured.  Preparetic  phases  are  theoretically  hopeful. 
Nor  is  it  so  certain  that  paretic  neurosyphilis  will  ultimately 
prove  a  perfectly  distinct  species  of  neurosyphilis.  General 


SUMMARY  AND  KEY  469 

paresis  seems  to  us  at  least  to  be  more  closely  related  to  diffuse 
neurosyphilis  than  is  tabes  dorsalis  to  diffuse  neurosyphilis. 
In  any  particular  case,  moreover,  during  a  good  part  of  the 
early  months  or  years,  it  is  difficult  or  impossible  to  tell  the 
paretic  from  the  non-paretic  forms  of  diffuse  neurosyphilis 
by  any  combination  of  clinical  observations  and  tests.  In 
the  instance  of  more  protracted  neurosyphilis,  e.g.,  tabetic, 
the  outlook  for  vascular  complications  is  such  that  antisyphi- 
litic  treatment  directed  at  prevention  of  these  complications 
is  scientifically  warrantable,  even  if  the  tabetic  process  itself 
proves  unassailable.  The  old  distinction  of  syphilis  and 
parasyphilis,  so  striking  and  apparently  satisfactory  when 
introduced  by  Fournier,  seems  to  be  a  false  distinction  which 
should  be  dropped.  Therapeutically,  we  should  approach 
all  cases  of  neurosyphilis  without  bias  or  nihilistic  pre judg- 
ments. 

(3)  RE  universal  applicability  of  syphilis  tests  in  nervous 
and  mental  cases. 

The  importance  of  putting  every  neurosis  or  psychosis 
through  syphilis  tests  is  not  based  alone  on  the  frequency  of 
neurosyphilis,  though  neurosyphilis  is  surely  frequent  enough. 
The  importance  of  universally  applying  these  tests  is  estab- 
lished by  the  experience  of  lingering  doubts  both  in  the  phy- 
sician's mind  and  (nowadays  increasingly)  in  the  patient's 
and  friends'  minds,  so  long  as  these  tests  are  not  applied. 
Nor  should  the  positive  serum  Wassermann  reaction  fail  to 
be  followed  by  lumbar  puncture  and  appropriate  tests.  The 
general  practitioner  confronting  neuroses  or  psychoses  —  and 
what  practitioner  does  not?  —  must  not  expect  valuable 
results  from  consultation  with  neurologists  and  psychiatrists 
when  he  does  not  carry  to  these  specialists  the  results  of  at 
least  the  serum  W.  R.  in  his  patient.  Not  only  are  prac- 
titioners, specialists,  and  patients  subject  to  discomfiture  on 
the  eventual  and  delayed  proof  of  syphilis  or  neurosyphilis, 
but  valuable  time  has  been  lost  to  treatment.  How  often 
the  physician  of  yore  (and  really  not  so  long  since)  had  to  be 
regarded  as  an  eccentric  virtuoso  if  he  tested  urine  as  routine! 
Well,  for  routine  use  in  nervous  and  mental  diseases,  the 
Wassermann  serum  reaction  is  at  least  as  important  as  urin- 


47O  SUMMARY  AND   KEY 

analysis.  Nor  would  we  cease  our  homily  with  the  general 
practitioner.  We  know  neurologists  and  psychiatrists  who 
use  the  Wassermann  test  only  when  it  is  likely  to  be  positive! 
But  they  are  dying  out. 


APPENDIX  A 

In  appendix  A  a  brief  outline  is  given  of  the  six  tests  (W.  R. 
on  blood  serum  and  spinal  fluid,  cell  count,  globulin  test, 
albumin  test,  gold  sol  test).  This  is  not  intended  as  a  com- 
plete working  manual  but  rather  as  indicating  the  methods 
used  in  diagnosis  in  the  cases  presented  herein.  For  more 
complete  details  the  reader  may  be  referred  to  textbooks 
on  the  subject  of  serology,  among  which  may  be  mentioned 
Kaplan:  "Serology  of  the  Nervous  System";  Plaut,  Rehm 
and  Schottmuller:  "  Leitfaden  zur  Untersuchungen  der 
Zerebrospinalfliissigkeit " ;  Kolmer:  "Infection,  Immunity 
and  Specific  Therapy,"  and,  for  the  Wassermann  technique, 
an  article  by  Dr.  W.  A.  Hinton  in  M.  J.  Rosenau's  "  Pre- 
ventive Medicine  and  Hygiene." 

Our  own  W.  R's.  have  been  performed  at  the  Wassermann 
laboratory  of  the  Massachusetts  State  Board  of  Health 
(formerly  the  Neuropathological  Testing  Laboratory,  Har- 
vard Medical  School),  under  the  supervision  of  Dr.  W.  A. 
Hinton.  The  other  tests  are  performed  at  the  Psychopathic 
Hospital.  It  is  very  important  that  a  close  relationship  should 
exist  between  the  clinician  and  the  Wassermann  laboratory 
if  the  most  is  to  be  obtained  from  the  reactions.  This  rela- 
tionship has  been  effectively  close  between  the  authors  and 
the  above-mentioned  laboratory;  and  has  enabled  us  to  get 
very  much  clearer  ideas  about  certain  cases  than  could  other- 
wise have  been  obtained. 

Cell  Count.  In  order  to  obtain  the  number  of  cells  per 
cmm.,  the  examination  should  be  made  of  the  fresh  fluid  as 
soon  as  possible  after  this  is  withdrawn.  The  most  con- 
venient counting  chamber  for  this  purpose  is  the  so-called 
Fuchs-Rosenthal  counting  chamber,  the  ruled  spaces  of  which 
contain  slightly  over  3  cmm.  (an  ordinary  blood  cell  counting 
chamber  may  be  used).  According  to  the  method  used  by 
us  the  cells  are  stained  in  a  pipette  with  Unna's  polychrome 
methylene  blue.  Using  a  white-counting  pipette,  stain  is 


472  APPENDIX  A 

drawn  up  to  the  first  or  second  marking  and  the  remainder  of 
the  pipette  filled  with  spinal  fluid.  This  makes  no  change 
in  the  dilution  for  practical  purposes.  After  two  or  three 
minutes  the  staining  is  satisfactory  and  the  counting  may 
be  done.  With  this  stain  a  differential  count  may  be  made. 
Plasma  cells  stain  a  lavender  as  contrasted  to  the  blue  of 
the  lymphocytes.  The  characteristic  halo  surrounding  the 
eccentric  nucleus  is  visible.  The  blood  cells  do  not  assume 
color  with  this  stain;  hence  it  is  unnecessary  to  add  any  acetic 
acid. 

For  permanent  preparations,  and  more  accurate  differ- 
ential counts  of  the  spinal  fluid,  the  Alzheimer  method  may 
be  used.  The  technique  is  given  in  a  paper  by  H.  A.  Cotton 
and  J.  B.  Ayer  as  follows:  * 

I.   Lumbar  puncture  in  the  usual  manner. 

2-  96%  alcohol,  in  proportion  to  twice  the  amount  of 
cerebrospinal  fluid,  is  added  drop  by  drop  and  well  mixed. 

3.  Centrifuge  the  mixture  for  one  hour  at  high  speed  in  a 
glass  tube  with  conical  end.     (An  ordinary  electric  urinary 
centrifuge  apparatus  can  be  employed,  the  tube  to  be  well 
stoppered  to  prevent  evaporation.) 

4.  The  supernatant  fluid  is  poured  off,  leaving  a  small 
coagulum  in  the  bottom  of  the  tube. 

5.  Add  absolute  alcohol  —  alcohol  and  ether  —  ether,  each 
separately  for  one  hour,  to  dehydrate  and  harden  coagulum. 

6.  The  coagulum  can  now  be  gently  loosened  from  the 
bottom  of  the  tube  by  a  long  needle.     The  tube  is  then  in- 
verted, and  the  coagulum  allowed  to  fall  into  the  hand  by  a 
quick  tap  on  the  end  of  the  tube.     Care  must  be  taken  not  to 
squeeze  or  handle  the  coagulum.     The  hand  is  placed  over 
a  small  homeopathic  vial,  containing  thin  celloidin,  and  the 
coagulum  allowed  to  drop  into  the  celloidin,  where  it  remains 
over  night  (twelve  hours  usually). 

7.  Coagulum  is  placed  in  thick  celloidin  which  is  allowed 
to  evaporate  slowly.     It  is  then  mounted  on  blocks  and 
sections  cut  14^  in  thickness. 

*  From  Mallory  and  Wright:  Manual  of  Laboratory  Tech- 
nique. 


APPENDIX  A  473 

8.  The  sections  are  stained  and  mounted  according  to 
the  following  procedure : 

(a)    Remove  celloidin  by  absolute  alcohol  and  ether. 
(6)    80%  alcohol. 

(c)  Water. 

(d)  Sections  are  carried  on  glass  or  platinum  needle  into 
a  dish  of  Pappenheim's  pyronin-methyl  green  stain  and  kept 
in  a  water-bath  at  40°  C.  five  to  seven  minutes. 

(e)  Quickly  cool  dish  in  running  water. 

(/)   Wash  off  superfluous  stain  in  plain  water. 
(g)   Absolute    alcohol    to    differentiate  —  until    no    more 
stain  comes  away  from  section. 
(h)    Clear  in  Bergamot  oil. 
(i)    Mount  in  balsam. 

The  normal  cell  count  may  be  stated  as  being  up  to  6  cells 
per  cmm.;  from  6  to  12  cells  may  be  considered  as  suggestive 
of  pathological  condition  and  more  than  12  cells  per  cmm.  as 
definitely  pathological.  The  type  of  cell  in  syphilitic  diseases 
is  preponderantly  the  small  lymphocyte.  A  low  percentage, 
that  is,  very  rarely  over  20%,  of  large  lymphocytes,  endo- 
thelial  phagocytic  cells,  polymorphonuclear  leucocytes  and 
plasma  cells  may  also  be  found.  The  finding  of  plasma 
cells  in  any  number  in  the  spinal  fluid  is  suggestive  although 
not  conclusive  evidence  for  the  diagnosis  of  paretic  neurosy- 
philis. 

Globulin  is  an  albumin  which  is  precipitated  by  half  satura- 
tion with  a  salt.  A  very  simple  and  satisfactory  test  is  known 
as  the  Nonne-Appelt  test,  which  has  been  modified  by  Ross- 
Jones.  Into  a  test-tube  of  small  diameter,  run  I  cc.  of  spinal 
fluid.  Place  under  this  fluid  with  a  pipette,  I  cc.  of  a  satu- 
rated solution  of  ammonium  sulphate  ((NH4)2SO4).  If  any 
globulin  is  present  a  white,  sharply-defined  ring  will  form  at 
the  junction  of  the  two  fluids.  According  to  our  readings,  a 
ring  that  is  just  visible  with  the  aid  of  a  black  background  is 
called  i  +  ,  a  ring  that  is  just  visible  without  the  black  back- 
ground, 2+;  a  ring  easily  perceptible,  3+  and  a  relatively 
very  heavy  ring,  4+.  On  shaking  the  tube,  if  globulin  is 
present,  the  fluid  will  show  turbescence. 


474  APPENDIX  A 

Another  simple  globulin  test  used  in  our  laboratory  as  a 
check  on  the  Nonne-Appelt  test  is  the  Pandy  test.  A  few 
cc.  of  a  clarified  10%  solution  of  phenol  are  placed  in  a  watch 
glass.  One  drop  of  spinal  fluid  is  run  into  this  solution.  A 
milky  turbescence  indicates  globulin. 

The  presence  of  globulin  in  the  spinal  fluid  is  always  an 
indication  of  abnormality  of  the  cerebrospinal  axis.  There 
is  nothing  differential  in  this  finding  as  it  occurs  in  all  inflam- 
matory processes.  However,  it  is  characteristically  present 
in  most  cases  of  neurosyphilis  (exception  to  the  rule:  the  pure 
vascular  type  does  not  show  globulin  in  a  very  high  per  cent) . 

Albumin  Test.  Albumin  in  small  quantities  is  present 
in  all  spinal  fluids.  Increase  over  the  normal  amount  occurs 
in  pathological  conditions 'such  as  most  cases  of  neurosyphi- 
lis, especially  in  those  in  which  globulin  is  found.  Any  al- 
bumin precipitant  may  be  used  for  rough  clinical  calculation, 
comparing  the  amount  of  precipitate  with  that  from  the  nor- 
mal fluid.  Our  method  is  to  place  I  cc.  of  spinal  fluid  in  a 
small  test  tube  of  about  5  mm.  diameter  and  to  precipitate  the 
albumin  by  the  addition  of  3  drops  of  33i%  of  trichloracetic 
acid.  This  test  has  its  chief  value  as  confirmatory  of  the 
globulin  test,  since  in  the  vast  majority  of  instances  where 
globulin  is  found  there  will  also  be  found  an  increase  in 
albumin. 

The  Gold  Sol  Reaction  is  an  empirical  test  discovered  by 
Carl  Lange  in  the  utilization  of  the  work  of  Zsigmondi  with 
solutions  of  colloidal  gold  and  albumins.  Briefly  the  details 
of  the  test  are  as  follows: 

Ten  tubes  are  set  up  in  a  rack.  To  the  first  tube  1 .8  cc.  of  a 
0.4%  of  salt  solution  is  added  and  to  each  of  the  following 
tubes  I  cc.  of  this  solution.  Then  to  the  first  tube  containing 
1.8  cc.  of  salt  solution  one  adds  0.2  cc.  of  the  spinal  fluid  to  be 
tested.  This  gives  a  dilution  of  I  to  10.  From  this  tube  i 
cc.  is  pipetted  into  the  second  tube  and  this  process  continued 
through  the  ten  tubes.  This  gives  dilutions  of  spinal  fluid 
of  i  to  i o,  I  to  20,  I  to  40,  etc.,  to  I  to  5120  in  the  last  tube. 
Then  5  cc.  of  colloidal  gold  solution  is  added  to  each  tube. 
A  positive  reaction  is  indicated  by  the  precipitation  or 
throwing  down  of  the  colloidal  gold  into  its  metallic  form. 


APPENDIX  A  475 

This  produces  a  change  in  color.  This  precipitation  may 
be  partial  or  complete  and  the  amount  of  precipitation  is 
indicated  by  the  color  and  is  read  as  follows: 

The  unchanged  fluid  is  called  o;  a  slight  change  giving  a 
red-blue  as  I ;  a  further  change  giving  a  blue-red  as  2 ;  a 
straight  blue  as  3 ;  a  lavender  or  violet  as  4 ;  and  the  colorless 
fluid  representing  complete  precipitation  as  5.  The  num- 
bers are  placed  in  a  row,  indicating  the  tube  in  which  the 
color  occurs.  The  fluid  from  a  case  of  paretic  neurosyphilis 
will  give  a  complete  precipitation  beginning  in  the  first  tube 
and  running  through  a  number  of  tubes  and  then  grading  off. 
It  may  be  indicated  5555431000.  The  characteristic 
reaction  of  fluids  from  tabetic  and  diffuse  neurosyphilis  is 
less  strong  than  from  the  paretic.  The  greater  part  of  the 
reaction  will  take  place,  however,  in  the  first  five  tubes,  but 
as  a  rule  it  will  not  begin  very  strongly  in  the  first  two. 
A  characteristic  reaction  is  1233210000.  Another 
reaction  that  may  be  considered  characteristic  of  the  tabetic 
or  diffuse  form  is  3332100000.  Fluids  from  non- 
syphilitic  cases  as  a  rule  give  a  reaction  having  its  greatest 
intensity  beyond  the  fifth  tube,  that  is,  in  the  high  dilutions. 

A  reaction  characteristic  of  brain  tumor  or  tuberculous 
meningitis  is  0000133210. 

The  conclusions  that  may  be  drawn  from  the  gold  sol 
reaction  have  been  summarized  by  one  of  the  authors  as 
follows : 

1.  Fluids  from  cases  of  general  paresis  will  give  a  strong 
and  fairly  characteristic  reaction,  especially  if  more  than  one 
sample  is  tested,  in  the  vast  majority  of  cases. 

2.  Very  rarely  a  general  paresis  fluid  will  give  a  reaction 
weaker  than  the  characteristic  one. 

3.  Fluids    from    cases    of    syphilitic    involvement    of    the 
central  nervous  system  other  than  general  paresis  often  give 
a  weaker  reaction  than  the  paretic,  but  in  a  fairly  high  per- 
centage of  cases  give  the  same  reaction  as  the  paretics. 

4.  Non-syphilitic  cases  may  give  the  same  reaction  as  the 
paretics;    these  cases  are  usually  chronic  inflammatory  con- 
ditions of  the  central  nervous  system. 

5.  When  a  syphilitic  fluid  does  not  give  the  strong  "  pa- 


476  APPENDIX  A 

retic  reaction,"  it  is  good  presumptive  evidence  that  the  case 
is  not  general  paresis;  and  this  test  offers  a  very  valuable 
differential  diagnostic  aid  between  general  paresis,  tabes  and 
cerebrospinal  syphilis. 

6.  The  term  "  syphilitic  zone  "  is  a  misnomer,  as  non- 
syphilitic  as  well  as  syphilitic  cases  give  reactions  in  this 
zone;  but  no  fluid  of  a  case  with  syphilitic  central  nervous 
system  disease  has  given  a  reaction  out  of  this  zone  (test 
thus  valuable  negatively).     Any  fluid  giving  a  reaction  out- 
side of  this  zone  may  be  considered  non-syphilitic. 

7.  Light  reactions  may  occur  without  any  evident  sig- 
nificance, while  a  reaction  of  no  greater  strength  may  mean 
marked  inflammatory  reaction. 

8.  Tuberculous    meningitis,   brain   tumor    and    purulent 
meningitis  fluids  characteristically,  though  not  invariably, 
give  reactions  in  higher  dilutions  than  syphilitic  fluids. 

9.  The  unsupplemented  gold  sol  test  is  insufficient  evi^- 
dence  on  which  to  make  any  diagnosis,  but  used  in  conjunc- 
tion with  the  Wassermann  reaction,  chemical  and  cytological 
examinations,   it   offers   much   information  looking   toward 
the  differential   diagnosis  of  general  paresis,   cerebrospinal 
syphilis,  tabes  dorsalis,  brain  tumor,  tuberculous  meningitis, 
purulent  meningitis. 

10.  We  believe  that  no  cerebrospinal  fluid  examination 
is  complete  for  clinical  purposes  without  the  gold  sol  test. 

The  Wassermann  reaction  as  carried  out  in  the  [Wasser- 
mann Laboratory  is  based  on  the  principles  of  the  original 
method  —  the  only  essential  modification  consists  in  the  em- 
ployment of  cholesterinized  alcoholic  extracts  of  human 
hearts  as  antigen  instead  of  aqueous  extracts  of  foetal  livers 
from  cases  of  congenital  syphilis.  Experience  has  shown  that 
properly  standardized  antigens  made  from  human  hearts  are 
much  more  sensitive  in  the  detection  of  true  cases  of  syphilis. 

Antigens.  Three  antigens  are  used,  each  being  an  alcoholic 
extract  of  human  heart  which  is  saturated  at  room  tempera- 
ture with  cholesterin.  These  antigens  differ  slightly  in  their 
sensitiveness.  Before  the  test  is  made  each  antigen  is  diluted 
with  0.85%  salt  solution  in  the  proportion  of  four  parts  of 


APPENDIX  A  477 

the  cholesterinized  antigen  extract  to  sixteen  parts  of  0.85% 
salt  solution.  The  amount  to  be  used,  the  dosage,  is  care- 
fully determined  by  testing  each  antigen  against  a  large 
number  of  known  positive  and  known  negative  specimens  of 
blood.  The  dosage  of  the  antigens  employed  is  less  than  one- 
half  the  amount  which  inhibits  hemolysis  when  the  antigen  is 
incubated  for  one  hour  with  the  hemolytic  system  which 
consists  of  complement,  amboceptor  and  cells  in  the  proper 
proportions.  These  antigens  are  designated  as  A,  B,  and  C. 
Antigen  A  is  the  most  sensitive.  B  and  C  are  very  similar 
to  each  other  quantitatively  and  qualitatively. 

Specimens  to  be  tested.  The  serum  which  separates  from 
the  clot  is  withdrawn,  centrifugalized  if  necessary,  and  then 
heated  at  55  degrees  for  thirty  minutes,  o.i  cc.  of  serum 
is  used  in  the  test  and  0.2  cc.  of  each  specimen  is  used  as 
a  control  to  exclude  the  presence  of  anti-complementary  sub- 
stances. Spinal  fluids  are  tested  in  two  ways.  As  a  routine 
0.5  cc.  of  the  spinal  fluid  is  used  in  the  test  and  i.o  cc.  is 
used  in  the  control ;  or  when  especially  requested  spinal  fluids 
are  titrated  by  using  respectively  i.o,  0.7,  0.5,  0.3,  and  o.i  cc. 
of  the  spinal  fluid  for  each  test  and  i.o  cc.  of  spinal  fluid 
for  the  control.  Spinal  fluids  are  not  inactivated. 

Complement.  The  complement  is  obtained  from  the  serum 
of  guinea  pig's  blood.  No  complement  is  used  when  older 
than  eighteen  hours.  A  10%  solution  and  0.85%  salt  solu- 
tion is  used  in  the  test.  The  amount  used  is  twice  the  mini- 
mum quantity  necessary  to  hemolyze  the  sensitized  cells. 

Sheep's  Corpuscles.  A  5%  suspension  of  sheep's  corpus- 
cles in  0.85%  salt  solution  is  prepared  from  defibrinated 
sheep's  blood.  The  corpuscles  are  washed  three  times  and 
for  each  washing  four  to  five  times  as  much  0.85%  salt 
solution  is  used  as  the  original  volume  of  the  defibrinated 
blood. 

Amboceptor.  The  amboceptor  is  prepared  by  injecting 
sheep's  corpuscles  into  a  rabbit.  The  serum  of  this  rabbit 
which  contains  amboceptor  is  diluted  with  0.85%  salt  solution 
so  that  0.25  cc.  will  hemolyze  0.5  cc.  of  a  5%  suspension  of 
sheep's  corpuscles.  In  the  test  twice  the  quantity  or  0.5  cc. 
of  amboceptor  is  used. 


478  APPENDIX   A 

Sensitized  Cells.  The  sensitized  cells  consist  of  equal 
parts  of  washed  sheep's  corpuscles  and  diluted  amboceptor. 
This  mixture  is  incubated  in  a  water  bath  at  37°  C.  for  a 
half  hour  to  effect  the  sensitization  of  the  cells. 

Technique  of  the  Wassermann  Test.  One-tenth  cubic 
centimeter  of  each  inactivated  specimen  of  serum  and  0.5  cc. 
of  each  uninactivated  specimen  of  spinal  fluid  is  pipetted  into 
a  separate  tube.  A  mixture  is  freshly  prepared  in  salt  solu- 
tion, each  cubic  centimeter  of  which  contains  the  proper 
amount  of  antigen  A  (the  most  sensitive  antigen),  and  two 
units  of  a  10%  solution  of  guinea  pig  serum  (complement). 
One  cubic  centimeter  of  this  mixture  is  pipetted  into  each 
test  tube.  These  tubes  are  then  incubated  for  forty  minutes 
in  a  water  bath  at  37°  C.  At  the  end  of  this  period,  sensi- 
tized cells  are  added,  and  the  tubes  are  again  incubated  in  a 
water  bath  at  37°  C.  for  one  hour.  Each  specimen  which 
shows  any  degree  of  inhibition  of  hemolysis  is  retested  in 
the  afternoon.  For  this  second  test  antigen  A  is  again  used 
and  in  addition  antigens  B  and  C.  A  control  is  also  made 
for  each  specimen  retested  to  eliminate  any  possibility  of 
the  inhibition  of  hemolysis  being  due  to  anti-complementary 
substances  in  the  serum  or  spinal  fluid  tested.  The  technique 
of  the  second  test  differs  in  no  wise  from  that  of  the  first, 
except  for  the  use  of  a  control  in  each  retested  specimen 
and  the  employment  of  three  antigens  instead  of  one.  The 
degree  of  positiveness  is  noted  for  each  retested  specimen  and 
compared  with  the  degree  of  positiveness  obtained  for  the  cor- 
responding specimen  with  the  same  antigen-complement-salt 
solution  mixture  in  the  morning's  test.  The  specimen  is  re- 
tested  on  the  next  day  when  discrepancies  occur  between  the 
morning  reading  for  antigen  A  and  the  afternoon  reading 
for  antigen  A.  From  the  above  description  it  will  be  noted 
that  the  negative  specimens  have  but  a  single  test  with  one 
antigen  only,  while  the  positive  specimens  are  retested,  thus 
permitting  a  confirmation  of  any  positive  reaction.  In  this 
way  attention  is  focalized  on  the  positive  specimens. 

Interpretation  of  Results.  Antigen  C  (the  weakest  of  the 
three  antigens)  is  used  entirely  for  diagnostic  purposes  and 
any  specimen  showing  the  slightest  degree  of  inhibition  with 


APPENDIX   A 


479 


this  antigen  and  stronger  degrees  of  inhibition  with  the  other 
antigens  is  reported  as  positive.  The  specimens  which  are 
strongly  or  moderately  positive  with  antigens  A  and  B  and 
negative  with  antigen  C  are  reported  as  doubtful.  In  testing 
spinal  fluids  by  the  titration  method,  antigen  C  is  used  and 
the  readings  are  based  upon  the  degree  of  inhibition  of  hemol- 
ysis  noted.  The  intensity  of  this  inhibition  is  indicated  by 
Arabic  numerals:  "  5  "  indicates  complete  inhibition, 
while  "  I  "  means  a  faint  cloudiness,  hence  a  weak  reaction. 
Intermediate  numbers  show  relative  intensity  varying  be- 
tween complete  inhibition  "  5  "  (strong  positive)  and  slight 
inhibition  "i  "  (weak  positive);  "— "  equals  no  inhibition 
(negative). 

Although  it  is  commonly  believed  that  the  recent  ad- 
ministration of  antisyphilitic  treatment  will  affect  the  reaction 
by  making  it  negative,  this  is  not  our  experience,  and  it  is, 
therefore,  not  necessary  that  treatment  be  withdrawn  for  a 
short  period  before  the  specimen  is  submitted  for  examination. 

The  reaction  as  carried  out  in  this  laboratory  has  the 
following  diagnostic  significance:  Positive  indicates  syphilis, 
except  very  rarely  in  acute  febrile  conditions  such  as  malaria 
and  pneumonia.  Negative  does  not  exclude  syphilis.  In 
obscure  conditions  a  series  of  less  than  three  negatives  has 
little  diagnostic  significance.  Doubtful  suggests  syphilis. 
It  is  therefore  advisable  to  submit  three  or  more  specimens 
in  such  a  case,  and  interpret  a  persistently  or  predominat- 
ingly doubtful  reaction  as  indicative  of  syphilitic  infection. 

Brack  Test.  A  new  serum  test  for  syphilis  has  recently 
been  described  by  C.  Bruck.*  Following  are  recent  results 
in  our  laboratory  with  this  test.f 

This  new  test  for  the  diagnosis  of  syphilis  by  C.  Bruck 
has  aroused  much  interest.  The  scientific  standing  of  Bruck 
and  the  simplicity  of  the  technique  led  us  to  overcome 
our  prejudice,  that  has  been  the  offspring  of  the  numerous 
tests  that  have  been  offered  of  late.  Bruck  states  that  since 


*  Bruck.     Munch,  med.  Wochen.  Jan.  22,  1917. 
f  Smith    and    Solomon.     Boston    Medical    and    Surgical 
Jour. 


480  APPENDIX  A 

the  discovery  of  the  complement  fixation  test  for  syphilis  by 
Wassermann,  Neisser  and  himself  in  1906,  he  has  been  trying 
to  find  a  simple  chemical  reaction  that  would  take  the  place 
of  the  complicated  technique  of  the  Wassermann  reaction. 
This  method,  as  he  has  published  it,  was  worked  out  and  is 
being  used  at  the  front,  in  the  present  war,  where  complete 
laboratory  equipment  is  not  available. 

Commencing  our  experiments  with  a  great  deal  of 
scepticism,  we  were  much  surprised  at  the  results  obtained, 
which  are  given  below.  Whatever  may  be  the  final  status 
of  the  test  in  the  determination  of  syphilis,  we  feel  that  there 
is  a  great  deal  of  interest  in  the  fact  that  this  simple  chemical 
reaction  does  pick  out  certain  differences  in  the .  composition 
of  blood  sera  and  that  apparently  a  large  number  of  syphilitic 
sera  differ  in  their  chemical  composition  percentage  from  the 
majority  of  non-syphilitic  sera. 

The  technique,  while  exceedingly  simple,  offers  many 
chances  for  errors  and  individual  variations  so  that  we  have 
thought  it  well  to  give  directions  and  cautions  at  some 
length. 

Bruck's*  technique  is  described  as  follows :  "The  test  is  made 
with  0.5  cc.  clear  serum  in  a  test  tube,  to  which  is  added  2  cc. 
of  distilled  water,  and  the  whole  shaken.  Then,  with  a 
precision  pipette,  0.3  cc.  of  the  ac.  nitr.  purum  of  the  German 
pharmacopeia  is  added  and  the  whole  thoroughly  shaken  and 
then  set  aside  at  room  temperature  for  ten  minutes.  Then 
1 6  cc.  of  distilled  water  at  room  temperature  is  added,  and 
closing  the  tube  with  the  finger,  it  is  shaken  up  and  down 
three  times  carefully,  not  vigorously  enough  to  make  it  foam. 
This  is  repeated  ten  minutes  later,  and  the  tube  is  then  set 
aside  for  half  an  hour.  By  this  time  the  precipitate  is 
entirely  dissolved  in  the  tube  with  the  normal  serum,  while 
the  syphilitic  serum  shows  a  distinct,  flocculent  turbidity. 
In  two  or  three  hours,  or  better  still,  in  twelve  hours,  the 
gelatinous  and  characteristic  precipitate  is  piled  up  on  the 
floor  of  the  test  tube." 

*  Bruck:  Journal  of  American  Medical  Association,  Vol. 
Iviii,  No.  12,  March  24,  1917,  p.  944. 


APPENDIX  A  481 

The  acid  is  prepared  by  diluting  the  Acidum  nitricum  of 
the  U.  S.  P.  (Sp.  gr.  1.403)  with  distilled  water  until  the 
hydrometer  shows  the  specific  gravity  1.149,  which  corre- 
sponds to  the  nitric  acid  of  the  German  pharmacopeia,  but 
since  this  requires  a  special  hydrometer,  a  simpler  method  is 
to  make  a  25  per  cent  solution  of  the  Acidum  nitricum,  which 
will  give  about  the  proper  specific  gravity. 

The  serum  is  obtained  by  allowing  10  cc.  of  blood  to  stand 
at  room  temperature  for  an  hour,  and  then  centrifuging. 
Serum  that  has  stood  for  some  time  may  be  used  as  well  as  the 
fresh,  and  even  bloody  serum  does  not  seem  to  confuse  the 
results  to  any  great  degree.  The  serum  gives  the  same 
results  with  or  without  inactivation.  Post-mortem  blood 
gave  results  as  constant  as  that  obtained  during  life,  in  the 
few  cases  that  we  had  in  this  series.  But  the  reaction  may 
be  influenced  markedly  by  the  size  of  the  test  tubes.  We 
have  found  that  the  13  X  1.9  cm.  is  the  most  favorable  size. 

When  one  first  thinks  of  this  test  it  appears  very  simple 
and  probably  somewhat  crude  as  a  chemical  reaction,  but 
there  are  certain  precautions  that  must  be  observed,  and 
several  hundred  normal  and  syphilitic  sera  should  be  tried 
before  the  investigator  can  feel  that  he  has  a  refined  routine 
technique.  There  is  the  personal  equation  which  must  be 
watched,  for  here  is  probably  the  greatest  source  of  error, 
and  readily  explains  why  two  different  persons  get  widely 
varying  results  with  the  same  sera  if  they  have  done  only  a 
few  dozen  tests.  We  must  take  it  for  granted  that  the 
reaction  is  a  quantitative  one,  where  some  positive  reactions 
may  differ  only  slightly  from  the  normal  non-syphilitic,  and, 
furthermore,  any  normal  serum  may  be  made  to  give  a  positive 
reaction,  and  almost  any  positive  serum  be  made  to  give  a 
negative  by  improper  manipulation  at  some  point  in  the  test. 
There  are  as  many  places  for  error  to  creep  in  as  there  are 
steps  in  the  process.  Bruck  has  omitted  many  details  in 
his  publication,  which  allow  personal  variations,  and  so  we 
have  tried  to  develop  a  routine  process  that  will  eliminate 
as  many  of  these  as  possible. 

We  shall  here  attempt  to  explain  the  methods  which  we 
have  found  most  satisfactory  and  at  the  same  time  indicate 


482  APPENDIX  A 

the  places  where  error  is  likely  to  occur.  The  0.5  cc.  of 
serum  is  added  to  2  cc.  of  distilled  water,  and  shaken  thor- 
oughly. Now  add  slowly  exactly  0.3  cc.  of  acid  from  a 
precision  pipette,  care  being  taken  it  does  not  flow  down  the 
side  of  the  tube.  The  tube  should  be  shaken  gently  while  the 
acid  is  being  added,  for  this  prevents  the  formation  of  a 
flocculent  precipitate  in  normal  serum  which  is  difficult  to 
dissolve  later.  After  the  acid  is  added  shake  each  tube 
gently  to  make  sure  that  these  flakes  do  not  persist.  It  is 
difficult  to  shake  each  tube  in  exactly  the  same  manner,  as 
must  be  done  if  we  expect  uniform  results. 

The  first  250  tests  of  this  series  were  made  by  allowing  the 
tubes  to  stand  for  ten  minutes  as  Bruck  advocates.  Then  we 
found  that  practically  all  sera  gave  a  positive  reaction  if 
allowed  to  stand  15-20  minutes,  and  so  in  the  other  tests  of 
the  series  an  attempt  was  made  to  make  the  reaction  more 
sensitive  by  allowing  the  tubes  to  stand  only  6-7  minutes. 
During  this  time  the  tubes  should  be  shaken  gently  once  or 
twice.  The  manner  in  which  the  16  cc.  of  water  is  added  also 
influences  the  reaction.  If  allowed  to  flow  freely  in  upon 
the  precipitate,  the  positive  may  be  forced  into  solution  as 
well  as  the  negative.  Both  pipette  and  tube  should  be 
slanted  and  the  water  allowed  to  flow  down  the  side  of  the 
tube  without  disturbing  the  precipitate.  If  all  has  gone  well 
up  to  this  point,  we  may  see  a  marked  difference  between  the 
normal  and  syphilitic  precipitates,  in  that  the  normal  will 
begin  to  go  into  solution  at  once,  thus  clouding  the  water, 
while  a  positive  precipitate  will  be  composed  of  large  flakes 
which  show  little  or  no  tendency  to  go  into  solution  or  cloud 
the  water  above.  It  must  be  remembered  that  the  most 
flocculent  positive  precipitate  will  go  into  solution  if  the  fluid 
is  splashed  or  shaken  too  hard  while  the  tube  is  being  inverted. 
If  any  doubt  as  to  the  character  of  the  precipitate  now  exists, 
it  may  be  allowed  to  stand  ten  minutes  longer,  and  again 
inverted  as  before,  or  even  repeated  several  times  during  the 
next  hour  or  two.  We  see  no  reason  why  the  tubes  should  be 
left  to  stand  over  night,  for  during  this  time  a  precipitate 
usually  settles  in  the  normal  tubes.  This,  however,  differs 
from  the  syphilitic  precipitate  in  that  it  is  still  finely  granular 


APPENDIX  A  483 

and  goes  back  into  solution  readily  when  the  tubes  are 
inverted. 

In  view  of  these  possible  grounds  for  error,  it  is  only 
logical  to  run  controls  of  known  positive  and  known  negative 
sera  along  with  each  group  of  unknown  bloods,  and  even  then 
certain  tubes  will  seem  doubtful,  in  which  event  the  test 
should  be  repeated  with  added  precaution  to  see  if  a  definite 
positive  or  negative  reaction  may  be  obtained. 

In  the  last  tests  of  this  series  we  seemed  to  aid  the  reaction 
by  rendering  the  serum-water  solution  alkaline  by  one  or  two 
drops  of  10  per  cent  potassium  hydroxide  before  the  acid  was 
added.  The  positive  sera  have  a  larger  precipitate,  while 
the  normal  seem  to  dissolve  more  readily. 

TABLE  I 
Syphilis:  nervous  system  involved. 

iWassermann  and  Bruck  agree  positively 47 

negatively 7 

"                       "         at  variance IO 

Tabes  Dorsalis   {  Wassermann  and  Bruck  agree  positively 3 

f  Wassermann  and  Bruck  agree  positively 8 

Cerebrospmal      j  „  „  negatively 3   ; 

Juvenile  Paresis  (  Wassermann  and  Bruck  agree  positively I 

Summary  Wassermann  and  Bruck  agree  positively 59 

"  negatively 10 

"  "        at  variance 10 

TABLE  II 

Syphilis:  nervous  system  not  involved. 

f Wassermann  and  Bruck  agree  positively 12 

»                       "         at  variance 5 

,    f  Wassermann  and  Bruck  agree  positively 3 

CongemtalSyph.j              „                       „              negatively 2. 

Summary:  Wassermann  and  Bruck  agree  positively 15 

"  negatively _2 

"  "        at  variance 5 

TABLE  III 

Non-syphilitic:  Wassermann  reaction  negative. 

Doubtful  or  positive  Bruck 86 

Bruck  test  negative 216 

Total  for  three  groups: 

Wassermann  and  Bruck  agree  positively 74 

"                     "                negatively 230 

!*                   "             at  variance 101 


484  APPENDIX  A 

The  tests  here  reported  were  made  on  blood  sera  obtained 
from  patients  admitted  to  the  Psychopathic  Hospital  and  its 
Out-Patient  Department.  As  a  routine  Wassermann  test 
is  made  on  each  patient  who  enters  the  hospital,  it  was  only 
necessary  to  take  another  tube  of  blood  from  each  patient, 
and  check  the  results  in  each  instance  with  the  Wasser- 
mann reaction.  As  it  takes  several  days  to  get  the  report 
from  the  Wassermann  laboratory  of  the  State  Board  of 
Health,  there  was  no  chance  of  being  prejudiced  by  a  previous 
knowledge  of  the  Wassermann  reaction.  The  cases  for  the 
most  part  were  those  of  mental  disease;  the  majority  in  good 
general  physical  health. 

A  comparison  of  the  total  number  with  the  Wassermann 
reaction  shows  that  there  was  a  general  agreement  of  304  of 
the  405  cases  tested,  or  a  percentage  agreement  of  practically 
75%.  In  considering  the  cases  of  syphilis  of  the  central 
nervous  system  in  a  group  by  themselves,  we  find  that  the 
agreement  is  closer,  since  69  of  the  79  cases  tested,  or  87% 
agreed  without  any  question  of  doubt.  It  will  be  noted  that 
in  several  cases  of  general  paresis,  the  Wassermann  reaction, 
which  was  repeated  at  intervals,  was  negative,  and  in  most  of 
these  cases  the  Bruck  test  was  negative  also.  Our  few  cases 
of  congenital  and  latent  syphilis  also  checked  very  closely 
with  the  Wassermann  test.  In  the  various  groups  of  mental 
cases  in  this  series,  no  factor  of  interference  was  discovered. 
It  is  also  of  interest  that  in  the  cases  where  the  blood  was 
obtained  post  mortem,  the  Bruck  test  agreed  with  the  Wasser- 
mann result  obtained  on  ante-mortem  blood  serum.  Further 
work  on  post-mortem  sera  will  be  reported.  Some  of  the 
patients  not  included  in  the  syphilitic  groups  that  have  a 
negative  Wassermann  and  no  clinical  signs  of  syphilis,  give  a 
history  of  previous  infection  at  some  time,  which  might  partly 
account  for  the  variations  in  the  two  tests. 

CONCLUSIONS 

I.  We  present  results  of  the  Bruck  sero-chemical  test  in  405 
cases.  In  101  of  these  cases  there  were  definite  clinical 
manifestations  of  syphilis,  in  which  the  Wassermann 
and  Bruck  tests  agreed  positively  in  74  or  75%.  The 


APPENDIX  A  485 

two  tests  agreed  negatively  in  12  instances,  and  were 
at  variance  in  15. 

2.  In  the  group  which  showed  syphilis  of  the  nervous  system 

we  had  64  cases  of  clinically  certain  general  paresis,  of 
which  the  Wassermann  and  Bruck  tests  agreed  in  54 
instances,  or  practically  85%.     In  other  forms  of  central 
nervous  system  involvement  the  agreement  was  100% 
in  the  15  cases  tested. 

3.  In  the  cases  with  no  apparent  involvement  of  the  nervous 

system  the  agreement  was  somewhat  less,  being  76%. 
This  may  be  in  keeping  with  the  fact  that  the  Wasser- 
mann test  was  not  so  strongly  positive  in  these  cases. 

4.  The  advantages  of  the  test  are:    (i)   the  short  time 

required  to  do  the  test;  (2)  the  limited  amount  of 
apparatus  necessary,  and  (3)  the  simplicity  of  the 
technique. 

5.  The  disadvantages  of  the  test  seem,  for  the  most  part, 

to  be  bound  up  in  the  personal  variations  that  are  apt 
to  occur. 

6.  We  are  here  dealing,  most  probably,  with  a  quantitative 

chemical  difference  in  the  protein  content  of  syphilitic 
and  non-syphilitic  sera,  the  nature  of  which  is  not 
understood  by  us.  It  is  our  hope  that  this  may  be 
brought  to  light  in  the  near  future  in  the  field  of  chem- 
istry. 


APPENDIX  B 

COMMON    METHODS    OF    TREATMENT    USED 
IN   CASES  OF  NEUROSYPHILIS 

The  treatment  for  neurosyphilis  according  to  the  viewpoint 
of  the  authors  is  treatment  for  syphilis.  It  is  necessary  in 
order  to  cure  a  case  of  neurosyphilis  to  cure  the  syphilis  in 
the  patient.  Accordingly,  the  methods  of  treatment  best 
adapted  for  the  cure  of  syphilis  are  indicated  in  the  treatment 
of  neurosyphilis.  As  experience  shows  that  it  is  often  more 
difficult  to  cure  the  neurosyphilitic  cases,  treatment  will  have 
to  be  pushed  with  greater  intensity  than  in  some  non-nervous 
system  syphilis.  In  general,  then,  the  methods  that  have 
been  applied  by  the  syphilologist  will  be  used  in  the  treat- 
ment of  cases  of  neurosyphilis.  In  addition,  methods  at- 
tempting to  bring  the  drug  into  local  contact  with  the  central 
nervous  system  have  been  devised.  The  methods  of  treat- 
ment have  been  in  part  indicated  in  Chart  27. 

The  method  chiefly  used  in  treatment  of  the  cases  of  this 
book  is  what  we  have  called  intensive  systematic  intravenous 
treatment.  The  treatment  consists  of  intravenous  injections 
of  salvarsan  (or  a  substitute  for  salvarsan,  as  arsenobenzol 
and  diarsenol)  given  in  a  dose  of  about  0.6  gram  and  repeated 
twice  a  week  over  a  period  of  a  number  of  months.  In 
addition,  injections  of  mercury  salicylate  averaging  0.065 
gram  once  a  week  are  given  and  potassium  iodid  by  mouth. 
As  indicated,  the  important  point  is  to  keep  up  treatment  for 
a  long  period  of  time.  This  method  has  produced  practically 
no  untoward  results,  certainly  no  more  untoward  results 
than  are  to  be  expected  with  salvarsan  in  smaller  quantities 
and  it  has  seemed  to  us  that  the  therapeutic  results  have 
been  as  satisfactory  as  in  any  other  form  of  treatment. 

Specialized  forms  of  treatment  intended  to  place  the  drug 
in  contact  with  the  central  nervous  system  may  be  described 

486 


APPENDIX  B  487 

under  the  headings  of  spinal  intradural  treatment  and  cere- 
bral subdural  and  intraventricular  treatment. 

Three  main  therapeutic  agents  have  been  largely  used. 
These  are  (i)  salvarsanized  serum  according  to  the  method 
of  Swift-Ellis  (in  vivo).  The  serum  according  to  this  method 
is  prepared  as  follows:  An  intravenous  injection  of  salvarsan 
is  given  to  a  patient  and  blood  withdrawn  at  the  end  of  one- 
half  hour.  This  is  allowed  to  clot.  The  serum  is  removed 
and  after  inactivation  at  56°  C.  for  one-half  hour  it  is  ready 
for  use.  The  average  dose  is  15  to  30  cc.  of  serum.  As  a 
matter  of  fact,  it  is  not  necessary  to  use  the  blood  serum  from 
the  same  patient  to  whom  the  intraspinous  injection  is  to  be 
given.  (2)  The  salvarsanized  serum  according  to  the  method 
of  Ogilvie  (in  vitro).  Blood  serum  is  prepared  from  any 
patient  and  to  it  is  added  salvarsan  in  such  a  strength  that 
the  amount  to  be  injected,  10  to  30  cc.  of  serum,  will  contain 
o.oooi  to  o.ooi  gm.  (3)  Mercurialized  serum  according  to 
the  method  of  Byrnes.  Mercury  bichloride  is  added  to  blood 
serum  in  such  proportion  that  the  amount  of  serum  to  be 
injected  will  contain  from  0.00065  gram  to  0.0026  gram. 

The  method  of  intraspinous  injection  is  to  perform  lumbar 
puncture,  withdraw  an  amount  of  fluid  approximately 
equivalent  to  the  amount  to  be  injected;  then  allow  the  serum 
to  be  injected  to  run  in  by  gravity. 

For  the  cerebral,  subdural  and  intraventricular  injections, 
the  same  sera  may  be  used  as  for  the  intraspinous.  Five  or  six 
times  as  much  salvarsan  may  be  given,  but  a  smaller  amount  of 
serum  may  be  advisable,  that  is,  10  to  15  cc.  To  perform  injec- 
tions a  trephine  opening  is  made  in  the  calvarium  about  the  size 
of  a  dime.  The  location  of  choice  for  the  opening  is  slightly 
back  of  the  longitudinal  prominence  just  to  the  right  of  the 
median  line,  to  avoid  the  frontal  sinus.  For  subdural  injec- 
tions a  curved  needle  is  thrust  between  the  dura  and  the 
brain  and  the  serum  allowed  to  flow  in  slowly  by  gravity.  For 
the  intraventricular  injections  a  blunted  spinal  puncture 
needle  is  thrust  through  the  brain  substance  into  the  3rd  ven- 
tricle. When  the  3rd  ventricle  is  reached  the  clear  cerebral 
fluid  will  flow  out ;  then  after  withdrawing  a  sufficient  amount, 
the  serum  may  be  introduced  by  gravity.  The  trephining  may 


488  APPENDIX  B 

be  done  under  local  anesthesia  but  as  a  rule  it  is  better  to 
induce  general  anesthesia.  The  subsequent  injections  can 
be  made  without  recourse  to  any  anesthesia  whatsoever,  as 
they  are  practically  painless. 

All  procedures  both  in  the  injections  and  in  the  preparation 
of  sera  are  naturally  to  be  performed  under  aseptic  conditions. 


INDEX 


Abscess,  tonsillar,  associated  with  neu- 

rosyphilis,  250. 
Addison's  disease  in  juvenile  paretic, 

279. 

Agraphia,  101. 
Albumin  test,  474. 
Allbutt,  Clifford,  257. 
Alcoholism,  chronic,  227. 
Alcoholic  dementia,  237. 

epilepsy,  229. 

hallucinosis,  225. 

pseudoparesis,  222,  223,  451. 
Allergie,  129,  204. 
Alzheimer,  428. 

method,  472. 
Amboceptor,  477. 
Amnesia,  195. 
Anaphylaxis,  129. 
Anatomical  formulae,  25. 
Antigens,  476. 
Aortic  aneurysm,  35,  439. 

sclerosis,  41,  46,  135. 
Aphasia,  31,  43,  101,  262,  445. 
Apoplexy,  197. 

Argyll- Robertson  pupil,  209,  212,  217, 
291,  450. 

as  isolated  symptom,  217. 

in  alcoholism,  214,  229. 
Arndt,  Junius  and,  249. 
Arsenobenzol,  375,  377,  389,  486. 
Arteriosclerosis,  cerebral,  101. 

not  a  contraindication  to  intensive 
salvarsan  therapy,  359. 

radial,  68. 

Ascending  lesion,  23. 
Asymmetrical  lesions,  19. 
Ataxia,  31,  223. 

Atheromatous  degeneration,  35. 
Atrophy,  cerebellar,  39. 

cerebral,  47,  134,  205. 

parenchymal,  41. 

pontine,  39. 


Atypical  case  congenital  neurosyphilis, 

270. 
Ayer,  J.  B.,  472. 

Ballet,  72. 

Barrett,  A.  M.,  54,  175,  187,  212,  218, 

219. 

Bechterew,  219. 
Binet  and  Simon,  304. 
Binet  scale,  277. 
Birnbaum,  403. 

Blood  pressure,  high,  70,  262,  124. 
Ely,  252. 

Bonhoeffer,  404,  415,  417. 
Bordet,  427. 
Bratz,  278. 
Bruck  test,  479. 
Bruck,  C.,  479. 
Bumke,  214. 

Canavan,  256. 
and  Southard,  70. 

Cell  count,  471. 

Cerebral   syphilis,   see   diffuse   neuro- 
syphilis. 

Cerebrospinal     syphilis,     see     diffuse 
neurosyphilis. 

Cervical    hypertrophic    meningitis    of 
Charcot,  56,  441. 

Chancre,  extragenital,  75,  342. 

Character  change,  neurosyphilis,  314. 

Charcot,  60,  186. 

Choroiditis,  242. 

Christian,  407. 

Cimbal,  403. 

Civilization  and  syphilis,  76. 

Clinical  evidences  of  syphilis,  131. 

Clouston,  158. 

Collins,  Joseph,  145. 

Compensation   in   neurosyphilis,   309, 
402,  456. 

Complement,  477. 


489 


490 


INDEX 


Conduct  disorder,  38. 
Congenital    syphilis,   absence  of  stig- 
mata, 318. 

as  cause  of  feeblemindedness,  159,447. 

involvement  of  nervous  system  in, 

274. 
Congenital  neurosyphilis,  270,  395. 

resembling  feeblemindedness,  272. 
Conjugal  neurosyphilis,  263. 
Convulsions,  43,  101,  248,  362. 

cause  of  in  paretic  neurosyphilis,  232. 

in      psychopathic      subject      with 

syphilis,  417. 

Corneal  opacity,  syphilitic,  234. 
Cotard,  73. 
Cotton,  H.  A.,  472. 
Craig,  C.  B.,  152,  196. 
Cramer,  125. 
Cranial  neurosyphilis,  140. 

tenderness,  139. 
Crises,  gastric,  367. 
Cysts,  ependymal,  59. 

of  softening,  27,  36,  54. 
Cytorrhyctes  luis,  381. 

Dana,  Charles  L.,  65,  77,  78. 

Dazed  states,  264. 

Deafness,  63. 

Decompression,  138. 

Defective  delinquent  —  diffuse  neuro- 
syphilis, 300,  455. 

Dejerine-Tinel,  61. 

Delinquency  and  juvenile  neurosyphilis, 
298. 

Delirium  tremens,  332. 

Dementia,  137. 

Dementia     paralytica,      see      paretic 
neurosyphilis. 

Dementia  praecox,  74,  185,  247. 

Depression,  95,  126. 

Depressive  drugs,  189. 

Diabetes,  and  neurosyphilis,  240. 
insipidus,  190. 

Diabetic  pseudoparesis,  238. 

Diarsenol,  377,  389,  391,  486. 

Differential  diagnosis,  alcoholism  and 

neurosyphilis,  227,  231,  234,  236. 
brain  tumor,  diabetic  pseudoparesis 

and  neurosyphilis,  238. 
diffuse    and    paretic    neurosyphilis, 
165,  193,  247. 


Differential  diagnosis,  manic-depressive 

psychosis  and  neurosyphilis,  69. 
multiple  sclerosis  and  neurosyphilis, 

253,  255. 
neurasthenia  and  neurosyphilis,  65, 

183. 
senile  arteriosclerotic  psychosis  and 

neurosyphilis,  262. 

Diffuse  neurosyphilis,  cerebrospinal 
syphilis,  cerebral  syphilis,  spinal 
syphilis,  17,  80,  85,  97,  103,  122, 
140,  183,  193,  300,  331,  342,  359, 

433,  439,  443- 

premonitory  symptoms,  342. 

prognosis,  80,  103,  124,  433,  443. 

spinal  fluid  findings  in,  348. 

symptoms,  99. 

treatment,  98,  103,  184,  302,  390. 

treatment,  results,  343. 
Diplopia,  50,  184,  253,  356. 

causes,  140. 
Donath,  401,  403. 
Drastich,  407. 
Duco  and  Blum,  403. 
Dupr6,  407. 
Dysdiadochokinesis,  231. 

Ehrlich,  184,  428,  429. 
Encephalitis,  27,  248. 

disseminated,  218. 
Endarteritis,  220. 
Ependymal  cysts,  59. 
Ependymitis,  40,  47,  49,  134. 
Epilepsy,  192. 

alcoholic,  229. 

brought  out  by  syphilis,  415. 

Jacksonian,  103. 

parasyphilitic,  194. 

relation  to  juvenile  neurosyphilis,  277. 

syphilitic,  103,  194. 

syphilogenic,  415. 
Epileptic  neurosis,  195. 
Erb's  syphilitic  spastic  paraplegia,  147. 

treatment  of,  148. 
Euphoria,  73. 
Excited  states,  95. 
Exner,  M.  J.,  416. 

Exophthalmic  goitre,  syphilitic  (?),  205. 
Extraocular  palsy,  140,  441. 
Eye  changes  in  neurosyphilis,  257. 
Eye  muscles,  paresis  of,  17,  5°- 


INDEX 


491 


Facial  paralysis,  53. 

Families  of  neurosyphilitics,  275,  316, 

318,  320,  373,  431,  457. 
Family  of  neurosyphilitic,  normal  look- 
ing, but  syphilitic,  318. 
Familial  syphilis,  299,  306. 
Farrar,  C.  B.,  411. 
Fearnsides,   Head   and,   21,  140,   150, 

193,  217,  374,  378. 
Feeblemindedness,  395. 

and  congenital  syphilis,  159. 
Fernald,  W.  E.,  159,  273,  396. 
Fildes,  Mclntosh  and,  129,  329. 
Focal  changes,  221. 

meningitis,  50. 

softenings,  pontine,  54. 
Fournier,  142,  222,  186,  194,  381. 
Franz,  357. 
Froissart,  413. 
Fugue,  hysterical,  264. 

Gamier,  407. 

General    paresis,   see    paretic   neuro- 

syphilis. 
Glands,  270. 

Gliosis,  39,  47,  49,  136,  180. 
Globulin,  229. 

tests,  473. 

Glycosuria,  238,  241. 
Goddard,  397. 
Gold  sol  reaction,  247,  474. 

in  brain  tumor,  too. 

paretic,  85,  98. 

paretic,  other  tests  negative,  383, 385. 

in  purulent  meningitis,  100. 

syphilitic,  85,  98,  345. 
Graham,  Thomas,  429. 
Grandiosity,  72,  295,  455. 
Graves,  W.  W.,  157. 
Grille,  407. 
Gross,  257. 

Gumma,  see  gummatous  neurosyphilis. 
Gumma  of  tonsil,  250. 
Gummatous  neurosyphilis,  53,  56,  137, 
138,  140,  221,  362,  438. 

Hallucinations,  53. 

in  paretic  neurosyphilis,  249. 
Hauptmann,  348. 

Head  and  Fearnsides,  21,  140,  150,  193, 
210,  217,  374,  387. 


Headache,  53,  63,  122,  247,  352. 

causes  of,  209. 
Hecht,  399. 

Hemianopsia  in  neurosyphilis,  242. 
Hemiplegia,  31,  45,  80,  122,  262,  360. 

causes  of,  389. 
Hemitremor,  197. 
Heredity,  neuropathic,  84. 
Herxheimer  reaction,  152. 
Heubner,  427,  428. 
Hinton,  W.  A.,  471. 
Huntington's  chorea,  258. 
Hutchinsonian  teeth,  45. 
Hydrocephalus,  134,  306. 
Hyperreflexia,  explanation  of,  233. 
Hypochondriacal  ideas,  133. 
Hysteria,  815,  301. 
Hysterical  symptoms,  18. 

Incontinence,  vesical  in  tabetic  neuro- 
syphilis, 144. 

rectal,  56. 

Incubation  period  of  neurosyphilis,  152. 
Infectiousness  of  neurosyphilis,  95. 
Insight,  95. 
Insomnia,  63. 

Intracranial  pressure,  139,  362. 
Intraspinal  lesions,  95. 
Intraspinous  therapy,  122,  366,  486. 

unpleasant  results  of,  366. 
Intraventricular  injections,  389,  487. 
Involution  melancholia,  187. 
Iodine,  untoward  results,  of,  363. 
Iritis,  17. 

Jarisch-Herxheimer  reaction,  72. 
Joffroy,  214. 

and  Mignot,  64. 
Junius  and  Arndt,  249. 
Juvenile  neurosyphilis,  438,  447. 

relation  to  epilepsy,  277. 
Juvenile  paresis,  see  juvenile   paretic 

neurosyphilis. 

Juvenile  paretic  neurosyphilis,  juvenile 
paresis,  45,  154,  157,  272,  275,  298, 
306,  440. 

age  of  onset,  158. 

and  Addison's  disease,  279. 

and  delinquency,  298. 

prognosis,  156,  158,  162,  273,  275. 

treatment,  154,  161,  278,  299. 


492 


INDEX 


Juvenile    paretic    neurosyphilis,    with 

initial  trauma,  306. 
congenital  amputation  of  toes  in,  158. 
Juvenile    tabetic    neurosyphilis,    161, 

447- 

Kaplan,  255,  471. 

Keraval,  257. 

Key,  427. 

Knee-jerks,  absence  of,  223. 

lively,  75. 

return  of,  24. 

Koefod,  Solomon  and,  243. 
Kolmer,  471. 
Kraepelin,  65,  66,  69,  88,  91,  95,  187, 

225,  249. 
Krafft-Ebing,  84. 

Laignel-Lavastine,  413. 
Lange,  C.,  428,  429,  474. 
Lancinating  pains,  92,  141. 
Lepine,  408,  413. 
Leptomeningitis,  47,  54,  135. 
Lewandowski,  210. 
Liability  of  paretic,  295. 
Lissauer's  paralysis,  38. 
Locomotor  ataxia,  see  tabetic  neuro- 
syphilis. 
Long,  418. 

Lucke,  Baldwin,  93,  144. 
Lues  maligna,  250,  452. 
Lumbar   puncture,   untoward   effects, 

352. 

treatment  of,  354. 
Liith,  278. 
Lymphocytosis,  23,  30,  40,  49. 

McDonagh,  381. 

Mclntosh,  Fildes  and,  129,  329. 

Malaria,  cerebral,  simulation  of  paretic 

neurosyphilis,  245. 
Mallory  and  Wright,  472. 
Manic-depressive  psychosis,  68,  71,  77, 

187,  202,  291,  384,  442. 
Marie,  Chatelin  and  Patrikios,  412. 
Marie,  408,  414. 
Martin,  E.  G.,  313. 
Massary,  de,  414. 
Mattauschek  and  Pilcz,  347. 
Medicolegal  and  Social,  454. 

period  of  paretic  neurosyphilis,  414. 


Meilhon,  407. 
Memory,  failing,  63. 
Meningitis  hypertrophica  cervicalis  of 
Charcot,  56. 

sympathica,  19. 

syphilitic,  103. 
Mercurialization,  98. 
Mercury,  58,  83,  85,  98,  148,  193,  235, 
376,  377,  389,  391,  395,  486. 

untoward  results  of,  363. 
Metasyphilis,  89. 
Metchnikoff  and  Roux,  427,  428. 
Microgyria,  occipital,  47. 
Mignot,  Joffroy  and,  64,  66. 
Migraine,  19. 
Mitchell,  H.  W.,  218. 
Moebius,  429. 

Mott,  F.  W.,  158,  257,  308,  396,  437. 
Multiple  sclerosis,  253,  256. 

relation  of  syphilis  to,  254. 

spinal  fluid  findings  in,  254. 
Muscular  atrophy,  149,  446. 

syphilitic    relation    to    amyotrophic 

lateral  sclerosis,  150. 
Muscular  weakness,  279. 
Myerson,  A.,  196. 

Nageotti,  428. 

Nausea,  63. 

Neisser,  399. 

Nerve  trunk  tenderness,  148,  234. 

Nervousness,  63. 

Nervous  indigestion,  63. 

Neurasthenia,  63,  183. 

Neuritis,  cranial,  51. 

optic,  365. 

root,  235. 

syphilitic,  235. 

Neurorecidive,  152,  153,  184,  196,  235. 
Neuroses,  relation  of  syphilis  to,  186. 
Neurosyphilis,  187,  238,  240,  242. 

aggravated  on  military  service,  404. 

atypical,  258,  346. 

atypical  case  resembling  hysterical 
fugue,  264. 

dates,  428. 

forms  of,  20,  21,  28,  29,  95. 

galloping,  328. 

history  cf,  427. 

incubation  period,  152. 

infectiousness  of,  95. 


INDEX 


493 


Neurosyphilis,  laboratory  findings  in, 
82. 

latent,  142,  203. 

lesions,  303. 

lighted  up  by  stress  of  military  serv- 
ice, 412. 

and  marriage,  319. 

prevention,  320. 

onset,  64. 

in  primary  stage,  1 86. 

in  secondary  stage,  185,  283,  390. 

in  secondary  stage,  prognosis,  390. 

in  secondary  stage,  treatment,  153. 

spinal,  23. 

and  the  war,  399,  466. 
Nissl-Alzheimer  method,  427. 
Noguchi,  381. 

and  Moore,  428,  429. 
Nonne,  82,  125,  152,  186,  195,  196,  214, 
216,  235,  254,  265. 

-Apelt  test,  473. 
Numbness,  56. 
Nystagmus,  45,  253,  256,  279. 

Obersteiner,  249. 
Occupation  neurosis,  312. 
Ogilvie  method,  487. 
Operation  for  gumma,  139. 
Optic  atrophy,  256. 

in  juvenile  paretic  neurosyphilis,  154. 
Optic   thalamus,    syphilitic   lesion   of, 

205. 

Osteitis,  syphilitic,  311. 
Ozena,  350. 

Pains,  31. 
Pandy  test,  474. 
Paralysis,  123. 

recovery  from,  342. 

of  respiration,  248. 
Paranoia,  syphilitic,  225. 
Paraphasia,  19,  43. 
Paraplegia,  26,  30. 
Parasyphilis,  89. 
Paresis  sine  paresi,  126,  186,  204,  303, 

445- 

Paresis,  see  paretic  neurosyphilis. 

Paretic  neurosyphilis,  dementia  para- 
lytica,  general  paresis,  softening  of 
the  brain,  37,  63,  68,  74,  78,  80,  85, 
97,  131,  188,  192,  197,  199,  202, 


227,  241,  262,  289,  295,  309,  314, 
323,  338,  372,  375,  377,  382,  384, 
386,  388,  392,  435,  440,  442. 

adjuvant  causes  of,  414. 

causing  social  complications,  289. 

causes  of  death  in,  197. 

course,  85. 

duration,  88. 

forms,  95. 

improvement,  377. 

incidence  among  officers,  407. 

incidence  among  soldiers,  402. 

lesions  of,  131. 

"lighted   up  "by  domestic  stress  in 
civil  life,  420. 

"lighted  up"  by  "gassing,"  414. 

mortality  from,  89. 

nomenclature,  88. 

onset,  192. 

pathology  of,  436. 

prognosis,  435,  444. 

symptoms,  90,  131. 

symptoms,  mental,  87. 

symptoms,  physical,  86. 

versus  diffuse  neurosyphilis,  165. 

versus  vascular  neurosyphilis,    169, 
172. 

with  very  marked  meningitis,  332. 

with  very  marked  brain  atrophy,  335. 

without  mental  symptoms,  315. 

traumatic  exacerbation,  310. 

traumatic  form,  308,  413. 

traumatic,  shell  shock,  401. 

treatment  of,  85,  370,  372,  377,  382, 
384,  386,  388,  392. 

treatment,  results  of,  351. 
Pensions  for  disabilities  resulting  from 

venereal  disease,  409. 
Pensions  for  neurosyphilis,  411. 
Peripheral  neurosyphilis,  19. 
Perivascular  infiltration,  41. 
Pernicious  anemia  with  spinal  symp- 
toms, 267. 

Petit  mal  attacks,  195. 
Pforringer,  6l. 
Phobia,  67. 

Pilcz,  Mattauschek  and,  347. 
Pitres  and  Marchand,  421,  424. 
Plaut,  249,  348,  428. 
Plaut,  Rehm  and  Schottmiiller,  471. 
Plasmocytosis,  40,  49,  55. 


494 


INDEX 


Pleocytosis,  23,  220,  247,  344. 

effect  of  antisyphilitic  treatment  on, 
244,  376. 

in  remissions,  243. 

significance  of,  243. 

spinal  fluid  otherwise  negative,  270. 
Polydipsia,  190. 
Polyuria,  190. 
Pontine  hemorrhage,  219. 

softening,  54. 
Posey  and  Spiller,  257. 
Potassium  iodid,  58,  85,  98,  193,  222, 

376,  377,  389,  486. 
Preparesis,  65,  77,  78. 
Prince,  Morton,  195. 
Psammoma,  213. 
Pseudoneurasthenia,  66. 
Pseudoparesis,  449. 

alcoholic,  222,  229,  451. 

diabetic,  238. 

senile,  263. 

shell  shock,  421. 

syphilitic,  223,  371. 
Pseudoparetic  neurosyphilis,  222. 
Pseudotabes,  shell-shock,  424. 
Psychogenic  neurosyphilis,  189. 
Psychographic  disturbance,  228. 
Psychopathic  personality,  302. 
Ptosis,  350. 
Pupillary  reaction,  changes  in,  261. 

signs,  69. 

Pupils,  Argyll-Robertson,   see   Argyll- 
Robertson  pupils. 

irregular,  79,  201. 

normally  reacting  in  paretic  neuro- 
syphilis, 199. 

sluggish  reaction  to  light,  188. 

stiff  as  isolated  symptom,  265. 
Purkinje  cells,  binucleate,  48. 
Putnam,  James  J.,  19,  56. 
Pyramidal  tract  lesion,  bilateral,  326. 

sclerosis,  44. 

Quadriplegia  in  juvenile  paretic  neuro- 
syphilis, 275. 
Quincke,  427,  428. 

Randsklerose,  24. 

Ravaut,  428. 

Ravaut,  Sicard,  Nageotti,  Widal,  428. 

Rayneau,  407,  413,  414. 


Recovery,  77. 

Recurrences,  70. 

Redlich,  403. 

Regis,  73. 

Remissions,  122,  435,  445. 

Retardation,  187. 

Retention  of  urine,  56. 

Retinitis,  hemorrhages,  365. 

Richards,  R.  L.,  402,  404,  406,  409. 

Robertson,  A.  R.,  59. 

Rod  cells,  226,  297. 

Romberg  sign,  141,  216,  279. 

Root  sciatica,  syphilitic,  418. 

Rosenau,  471. 

Ross-Jones  test,  473. 

"  Rum  fit,"  229. 

Ryder,  Charles  T.f  42. 

Saddle-shaped  nose,  210. 
Salivation,  98. 
Salmon,  Thomas  W.,  89. 
Salvarsan,  75,  83,  85,   193,  222,  377, 
389,  486. 

provocative,  78,  79. 

untoward  results  of,  363. 
Salvarsanized  serum,  75. 
Schaudinn,  427,  429. 
Sciatic  pain  in  neurosyphilis,  149. 
Seizures,  31,  64,  83,  103,  444. 

causes  of  in  paretic  neurosyphilis, 
194. 

Jacksonian,  392. 

minor,  392. 

Senile  arteriosclerotic  psychosis,  262. 
Sensitized  cells,  478. 
Serieux  and  Ducaste,  96. 
Shaikewicz,  404. 
Shanahan,  278. 
Sheep's  corpuscles,  477. 
Shock,  42,  81. 
Sicard,  428. 
Six  tests,  80,  85. 

in  tabetic  neurosyphilis,  141. 
Smith  and  Solomon,  479. 
Social  cases,  454. 

service,  232. 
Solomon,  142,  255. 

and  Koefod,  243. 

Smith  and,  479. 

Southard  and,  202,  303. 
Somnolence,  45. 


INDEX 


495 


Southard,  E.  E.,  48,  134,  212. 

and  Canavan,  70. 

and  Solomon,  202,  303. 

and  Taft,  397. 
Spasms,  clonic,  326. 
Spastic   hemiplegia  in  paretic   neuro- 

syphilis,  323. 

Spastic  paraplegia,  Erb's,  147,  306. 
Spasticity,  18,  256. 
Speech  defect,  69,  133. 
Spiller,  150. 

Posey  and,  257. 

Spinal  fluid  findings  in  secondary  stage 
of  syphilis,  151,  185,  283. 

in  juvenile  paretic  neurosyphilis,  275. 

negative  in  diffuse  neurosyphilis,  140. 

negative     in     gummatous     neuro- 
syphilis, 138. 

negative  in  neurosyphilis,  216. 

negative    in    tabetic    neurosyphilis, 
269. 

in  tabetic  neurosyphilis,  141. 
Spinal  fluid,  withdrawal  for  therapeutic 

purposes,  377,  379. 

Spinal    syphilis,    see     diffuse    neuro- 
syphilis. 
Spirochetes,  "drug  fastness,"  381,  394. 

strains,  76,  263,  276,  381,  394. 
Steida,  405. 

Sterility  in  tabetic  neurosyphilis,  144. 
Stier,  407. 

Stokes,  Wile  and,  186. 
Suicide,  92,  126,  240,  296,  301. 
Summary,  427. 

Syphilis  aggravated  by  service,  406, 
411. 

on  service,  409. 
Syphilis  as  cause  of  diabetes,  241. 

as  cause  of  feeblemindedness,  396. 

hereditaria  tarda,  160,  318. 

history  of,  427. 

lesions  in,  329. 

of  lung,  211. 

from  Mongolian,  76. 

primary,  65. 

secondary,  65. 

tertiary,  lesions  in,  329. 
Syphilitic  feeblemindedness,  pathology 
of,  1 60. 

neuritis,  312. 

psychosis,  91. 


Syphilophobia,  67,  361. 
Syphilotoxins,  72. 
Swift,  129,  212. 
Swift  and  Ellis,  428,  429. 
method,  428,  487. 

Tabes     dorsalis,    see    tabetic    neuro- 
syphilis. 

Tabetic  neurosyphilis,  tabes  dorsalis, 
locomotor  ataxia,  30,  31,  141,  146, 
366,  367,  434,  446. 
associated  with  cerebral  symptoms, 

177- 

atypical,  143. 
cervical,  146. 
course,  141. 
with  negative  spinal  fluid  findings, 

269. 

prognosis,  94. 
shell  shock,  403. 

"shell  shocked"  into  paretic  neuro- 
syphilis, 401. 
symptoms,  93. 

symptoms  in  order  of  frequency,  145. 
treatment,  145,  366,  367. 
plus  vascular  neurosyphilis,  175. 
with  vascular  insult,  30,  439. 
versus  pernicious  anemia,  267. 
Taboparesis,  see    Taboparetic    neuro- 
syphilis. 
Taboparetic  neurosyphilis,  taboparesis, 

92,  135,  195,  284,  443. 
course,  92. 
nomenclature,  94. 
prognosis,  92,  443. 
and  typhoid  meningitis,  284. 
Taft,  A.  E.,  Southard,  E.  E.,  and, 
Talon,  407. 
Taylor,  E.  W.,  50. 
Temperature,  paretic,  376. 
Tests,  changes  under  treatment,  102. 
changed  to  negative  in  paretic  neuro- 
syphilis without  clinical  improve- 
ment, 385. 

changed  to  less  strongly  positive  in 
paretic  neurosyphilis  without  clini- 
cal improvement,  386. 
Therapeutic  conception,  324. 
Thibierge,  399. 
Thierry,  158. 
Throbbing  in  head,  63. 


496 


INDEX 


Thrombosis,  cerebral,  36,  42,  342,  357, 

360,  124. 

Thymus,  persistent,  282. 
Tibial  exostoses,  100. 
Tigges'  formula,  248. 
Todd,  J.  L.,  406,  409. 
Transient  deafness,  1 8. 
blindness,  18. 
paralysis,  124. 
paralysis,  condition  in  which  occurs, 

123. 
Trauma    and    juvenile    neurosyphilis, 

278,  306. 

neurosyphilis,  456. 
paretic  neurosyphilis,  199,  308,  310. 
syphilitic  osteitis,  311. 
Treatment  of  neurosyphilis,  67,  75,  83, 
124,  148,  184,  222,  235,  299,  328, 
332,  335,  342,  346,  350,  351,  355, 
359,  365,  366,  370,  372,  375,  382, 
384,  390,  392,  395,  419,  439,  457- 
case   in   which   theoretically   of    no 

avail,  323. 
methods,  356,  486. 

Treatment  of  syphilis,   effect  on  de- 
velopment of  neurosyphilis,   142, 

347- 
Tremor,  197. 

intention,  256. 
Tubercle,  80. 

Tuberous  sclerosis  of  Bourneville,  47. 
Tumor,  cerebral,  53,  191,  238,  253. 

pineal,  213. 

Unconsciousness,  53. . 
causes  of,  389. 

Vascular  changes,  220. 
Vascular  neurosyphilis,  31,42,  72,  296, 
359,  433,  440- 


Vascular    neurosyphflis,    plus    tabetic 
neurosyphilis,  175. 

prognosis,  433. 

versus  paretic  neurosyphilis,  169, 172. 
Veeder,  B.  S.f  274. 
Vertigo,  122. 
Viet,  278. 
Virchow,  427,  428. 
Vomiting,  53,  63. 

Warthin,  241. 
Wassermann  reaction,  191. 

and  alcoholism,  230. 

in  congenital  syphilis,  160,  271. 

meaning  of  "doubtful,"  360. 

negative  in  diffuse  neurosyphilis,  184. 

negative  in  juvenile  paretic  neuro- 
syphilis, 298. 

negative   in   spinal    fluid    in   spinal 
syphilis,  148. 

negative   in   spinal   fluid   in   neuro- 
syphilis, 101. 

negative  in  neurosyphilis,  252. 

negative  in  paretic  neurosyphilis,  77. 

technique,  476. 

titrations  in  spinal  fluid,  348. 
Wassermann,  Neisser  and  Bruck,  428. 
Weiler,  214. 
Weygandt,  403,  404. 
Widal,  Sicard,  Ravaut,  428. 
Wiles  and  Stokes,  186. 
Word  deafness,  35,  43. 

X-ray  diagnosis  of  bone  conditions,  136. 
Yerkes-Bridges,  304. 

Ziehen,  409. 
Zsigmondi,  429,  474. 


University  of  California 

SOUTHERN  REGIONAL  LIBRARY  FACILITY 

Return  this  material  to  the  library 

from  which  it  was  borrowed. 


BlOMtD 


FEB  1  3  1990 
REC'D 


3I 


THW 


3  1158  00260  3040 


